DRAFT

Question 1

– continuous, regulated process of blood cell production that includes cell renewal, proliferation, differentiation, and maturation.

Answer 1

Hematopoiesis

Question 2

Hematopoiesis Types:

Answer 2

Primitive hematopoiesis Definitive hematopoiesis

Question 3

Site of hematopoiesis:

Answer 3

ribs
sternum
skull
scapula
vertebrae
pelvic bones
proximal ends of the long bones

Question 4

Yolk sac

Answer 4

Mesoblastic phase

Question 5

Liver (main), spleen

Answer 5

Hepatic phase

Question 6

Bone marrow

Answer 6

Myeloid phase

Question 7

Gower I, Gower II, Portland

Answer 7

Mesoblastic phase

Question 8

Hb F (major), Hb A1, Hb A2

Answer 8

Hepatic phase

Question 9

Hb A1 (major), Hb A2, Hb F

Answer 9

Myeloid phase

Question 10

Primitive erythroblasts

Answer 10

Mesoblastic phase

Question 11

Erythroblasts Granulocytes Monocytes Megakaryocytes

Answer 11

Hepatic phase

Question 12

All blood cells

Answer 12

Myeloid phase

Question 13

Start: 19-20th day
End: 8-12th week

Answer 13

Mesoblastic phase

Question 14

Start: 5th to 7th week
End: 1st to 2nd week (after birth)

Answer 14

Hepatic phase

Question 15

Start: 5th month of gestation *Lifetime

Answer 15

Myeloid phase

Question 16

the spleen, liver, and the lymph nodes revert back to produce immature blood cells in certain abnormal conditions where the bone marrow cannot produce sufficient number of hematopoietic cells

Answer 16

extramedullary hematopoiesis

Question 17

are frequently noted on physical examination

Answer 17

✓ Hepatomegaly and splenomegaly

Question 18

Hematopoietic hormones

Answer 18

▪ Erythropoietin
▪ Thrombopoietin

Question 19

− produced by the kidneys (90%) and the liver (10%)

Answer 19

▪ Erythropoietin

Question 20

prevents the apoptosis of erythroid precursors

Answer 20

▪ Erythropoietin

Question 21

Stimulates Hb synthesis

Answer 21

▪ Erythropoietin

Question 22

Serves as differentiation factor causing the CFU-E to differentiate into pronormoblasts

Answer 22

▪ Erythropoietin

Question 23

− Also known as mpL kit ligand

Answer 23

▪ Thrombopoietin

Question 24

− Synthesized by the liver

Answer 24

▪ Thrombopoietin

Question 25

Note: the primary source of erythropoietin among the newborns is the

Answer 25

liver

Question 26

Adult Hematopoietic tissue

Answer 26

▪ Bone marrow ▪ Liver ▪ Spleen

Question 27

− hematopoietically active marrow

Answer 27

 Red marrow

Question 28

− consists of developing blood cells and their progenitors

Answer 28

 Red marrow

Question 29

− hematopoietically inactive

Answer 29

 Yellow marrow

Question 30

− composed primarily of adipocytes

Answer 30

 Yellow marrow

Question 31

− under physiologic stress, it will revert back to active marrow

Answer 31

 Yellow marrow

Question 32

: the process of replacing the active marrow by adipocytes

Answer 32

Retrogression

Question 33

• the primary site of hematopoiesis during the hepatic phase of hematopoiesis

Answer 33

▪ Liver

Question 34

Responsible for splenic culling/pitting of RBCs

Answer 34

▪ Spleen

Question 35

Removes senescent RBCs Stores

Answer 35

▪ Spleen

Question 36

1/3 of platelets

Answer 36

▪ Spleen

Question 37

▪ Refers to RBC production

Answer 37

Erythropoiesis

Question 38

Erythropoiesis
▪ Occurs in distinct anatomical sites called erythropoietic islands where each island consists of a macrophage surrounded by a cluster of erythroblasts

Answer 38

(suckling pig phenomenon)

Question 39

− a condition where oxygen content decreases within the tissues

Answer 39

▪ Tissue hypoxia

Question 40

− primary stimulus for the production of RBCs

Answer 40

▪ Tissue hypoxia

Question 41

− produces a dramatic increase in the production of EPO

Answer 41

▪ Tissue hypoxia

Question 42

Erythroblastic

Answer 42

Proerythroblast
Basophilic erythroblast
Polychromatophilic erythroblast
Orthochromic erythroblast
Polychromatophilic erythrocyte/Reticulocyte
Erythrocyte

Question 43

Rubriblastic

Answer 43

Rubriblast
Prorubricyte
Rubricyte
Metarubricyte
Polychromatophilic erythrocyte/Reticulocyte
Erythrocyte

Question 44

Normoblastic

Answer 44

Pronormoblast
Basophilic normoblast
Polychromatophilic normoblast
Orthochromic normoblast
Polychromatophilic erythrocyte/Reticulocyte
Erythrocyte

Question 45

RBC Maturation Series:

Question 46

▪ Earliest recognizable RBC precursor

Answer 46

Pronormoblast

Question 47

▪ Fine and uniform chromatin pattern

Answer 47

Pronormoblast

Question 48

▪ It takes approximately 3 days for the pronormoblast to develop into orthochromic normoblast

Answer 48

Pronormoblast

Question 49

▪ Nuclear chromatin becomes more clumped

Answer 49

Basophilic normoblast

Question 50

▪ Last stage with nucleolus (last stage of RNA synthesis)

Answer 50

Basophilic normoblast

Question 51

▪ Note: Hb is produced in this stage but not detected in light microscopy but in EM (Rodak)

Answer 51

Basophilic normoblast

Question 52

▪ Hb production begins during this stage

Answer 52

Polychromatophilic normoblast

Question 53

▪ Characterized by muddy, light gray appearance of cell due to variable amounts of pink coloration mixed w/ basophilia

Answer 53

Polychromatophilic normoblast

Question 54

▪ Last stage capable of mitosis

Answer 54

Polychromatophilic normoblast

Question 55

▪ Nucleus is tightly condensed and described as pyknotic

Answer 55

Orthochromic normoblast

Question 56

▪ Last stage w/ nucleus

Answer 56

Orthochromic normoblast

Question 57

▪ Part of this stage occurs in the bone marrow (2 days), and the later part of this stage takes place in the circulation (1 day)

Answer 57

Polychromatophilic erythrocyte

Question 58

▪ Anucleate

Answer 58

Polychromatophilic erythrocyte

Question 59

▪ When stained with supravital stain = reticulocyte

Answer 59

Polychromatophilic erythrocyte

Question 60

▪ Last stage capable of Hb synthesis

Answer 60

Polychromatophilic erythrocyte

Question 61

Earliest recognizable precursor

Answer 61

Pronormoblast/Proerythroblast/Rubriblast

Question 62

Last stage capable of mitosis

Answer 62

Polychromatophilic normoblast/Polychromatophilic erythroblast/Rubricyte

Question 63

Last stage with a nucleolus

Answer 63

Basophilic normoblast/Prorubricyte

Question 64

Last stage with nucleus

Answer 64

Orthochromic normoblast/Metarubricyte

Question 65

Last stage that can synthesize hemoglobin

Answer 65

Reticulocyte

Question 66

Caused by increased number of reticulocytes that are prematurely released from the bone marrow under the stimulus of EPO because of certain conditions (e.g., hypoxia, acute bleeding)

Answer 66

Stress or shift retics

Question 67

An elevated reticulocyte count accompanied w/ a shortened RBC survival

Answer 67

Polychromatophilia/Reticulocytosis

Question 68

▪ Anaerobic glycolysis

Answer 68

Embden-Meyerhof pathway

Question 69

▪ Supplies 90-95% ATP

Answer 69

Embden-Meyerhof pathway

Question 70

▪ G6PD and glutathione are generated in this pathway

Answer 70

Hexose Monophosphate Shunt

Question 71

▪ Purpose: prevents oxidative denaturation of hemoglobin

Answer 71

Hexose Monophosphate Shunt

Question 72

▪ Maintains the iron present in the Hb in a functional reduced state (Ferrous iron) for oxygen transport

Answer 72

Methemoglobin Reductase pathway

Question 73

▪ Generates 2,3-DPG

Answer 73

Rapoport-Luebering shunt

Question 74

▪ Alcohol intoxication

Answer 74

Acanthocyte Thorny cells Spur cell Spike cell

Question 75

▪ PK deficiency

Answer 75

Acanthocyte Thorny cells Spur cell Spike cell

Question 76

▪ Congenital abetalipoproteinemia (neuroacanthocytosis)

Answer 76

Acanthocyte Thorny cells Spur cell Spike cell

Question 77

▪ Severe liver disease (spur cell anemia)

Answer 77

Acanthocyte Thorny cells Spur cell Spike cell

Question 78

▪ Vitamin E deficiency

Answer 78

Acanthocyte Thorny cells Spur cell Spike cell

Question 79

▪ Lipid metabolism disorder

Answer 79

Acanthocyte Thorny cells Spur cell Spike cell

Question 80

▪ Hereditary stomatocytosis

Answer 80

Stomatocytes Mouth cell

Question 81

▪ Electrolyte imbalance

Answer 81

Stomatocytes Mouth cell

Question 82

▪ Liver disease, alcoholism

Answer 82

Stomatocytes Mouth cell

Question 83

▪ Rh null syndrome

Answer 83

Stomatocytes Mouth cell

Question 84

▪ Hydroxyurea therapy

Answer 84

Stomatocytes Mouth cell

Question 85

▪ Hemoglobinopathies (Hb CC, Hb SS, Hb SC)

Answer 85

Target cells Codocyte Mexican hat cell Platycyte Leptocyte Greek helmet cell Bull’s eye cell

Question 86

▪ Liver disease (flattened surface)

Answer 86

Target cells Codocyte Mexican hat cell Platycyte Leptocyte Greek helmet cell Bull’s eye cell

Question 87

▪ MDS

Answer 87

Ovalocytes/Elliptocytes Egg shape

Question 88

▪ Thalassemia

Answer 88

Ovalocytes/Elliptocytes Egg shape

Question 89

▪ Megaloblastic process

Answer 89

Ovalocytes/Elliptocytes Egg shape

Question 90

▪ IDA

Answer 90

Ovalocytes/Elliptocytes Pencil shape

Question 91

▪ Hereditary elliptocytosis

Answer 91

Ovalocytes/Elliptocytes Pencil shape

Question 92

▪ Idiopathic myelofibrosis

Answer 92

Ovalocytes/Elliptocytes Pencil shape

Question 93

▪ associated with hemolytic process

Answer 93

Spherocytes Bronze cell

Question 94

▪ ABO HDN

Answer 94

Spherocytes Bronze cell

Question 95

▪ Immune hemolytic anemia

Answer 95

Spherocytes Bronze cell

Question 96

▪ Hereditary spherocytosis

Answer 96

Spherocytes Bronze cell

Question 97

▪ Severe burns

Answer 97

Spherocytes Bronze cell

Question 98

▪ Others: normal aging process, storage phenomenon

Answer 98

Spherocytes Bronze cell

Question 99

▪ Artifact

Answer 99

Echinocytes (Evenly distributed uniformly sized blunt)

Question 100

▪ Seen in old specimen

Answer 100

Echinocytes (Evenly distributed uniformly sized blunt)

Question 101

▪ Associated w/ renal insufficiency

Answer 101

Burr cells (Irregularly sized and unevenly spaced spicules)

Question 102

▪ Dehydration

Answer 102

Burr cells (Irregularly sized and unevenly spaced spicules)

Question 103

▪ Azotemia

Answer 103

Burr cells (Irregularly sized and unevenly spaced spicules)

Question 104

▪ Hallmark of hemolytic anemia

Answer 104

Schistocytes Schizocytes Helmet cells Triangular cells Keratocytes

Question 105

▪ MAHA (DIC, TTP, HUS)

Answer 105

Schistocytes Schizocytes Helmet cells Triangular cells Keratocytes

Question 106

▪ Exposure of RBCs to heat or mechanical trauma

Answer 106

Schistocytes Schizocytes Helmet cells Triangular cells Keratocytes

Question 107

▪ Prosthetic heart valve

Answer 107

Schistocytes Schizocytes Helmet cells Triangular cells Keratocytes

Question 108

▪ Clostridial infection

Answer 108

Schistocytes Schizocytes Helmet cells Triangular cells Keratocytes

Question 109

▪ Removal of Heinz bodies

Answer 109

Keratocytes Bite cell Degmacyte Horned cell

Question 110

▪ Seen in G6PD deficiency

Answer 110

Keratocytes Bite cell Degmacyte Horned cell

Question 111

▪ Overt hemolysis

Answer 111

Semilunar bodies

Question 112

▪ Associated with malaria

Answer 112

Semilunar bodies

Question 113

▪ Myelofibrosis w/ myeloid metaplasia

Answer 113

Teardrop cells Dacryocytes

Question 114

▪ Myelophthisic anemia

Answer 114

Teardrop cells Dacryocytes

Question 115

▪ Pernicious anemia

Answer 115

Teardrop cells Dacryocytes

Question 116

▪ Beta-Thalassemia

Answer 116

Teardrop cells Dacryocytes

Question 117

▪ Hypersplenism

Answer 117

Teardrop cells Dacryocytes

Question 118

▪ Severe burns

Answer 118

Pyropoikilocytes

Question 119

▪ Hereditary pyropoikilocytosis

Answer 119

Pyropoikilocytes

Question 120

▪ Sickle cell anemia

Answer 120

Drepanocyte Sickle cell

Question 121

▪ Hb SC disease

Answer 121

Drepanocyte Sickle cell

Question 122

▪ Hb C disease

Answer 122

Folded cell Biscuit cell

Question 123

▪ Hb SC disease

Answer 123

Folded cell Biscuit cell

Question 124

Small round reddish-blue fragments of nucleus

Answer 124

Howell-Jolly bodies

Question 125

(+) Feulgen stain

Answer 125

Howell-Jolly bodies

Question 126

DNA

Answer 126

Howell-Jolly bodies

Question 127

Accelerated or abnormal erythropoiesis
Megaloblastic anemia
Alcoholism

Answer 127

Howell-Jolly bodies

Question 128

Reddish violet, thin ringlike, figure of eight, loop-shaped appearance

Answer 128

Cabot rings

Question 129

Mitotic spindle

Answer 129

Cabot rings

Question 130

Megaloblastic anemia
Lead poisoning
Homozygous thalassemia
Severe anemia

Answer 130

Cabot rings

Question 131

Small faint basophilic coccoid bodies near the periphery of RBCs

Answer 131

Pappenheimer bodies (Wright’s stain)
Siderotic granules (Prussian blue)

Question 132

Iron

Answer 132

Pappenheimer bodies (Wright’s stain)
Siderotic granules (Prussian blue)

Question 133

Refractory anemia
Sideroblastic anemia
Iron overload (hemosiderosis, hemochromatosis)
Thalassemia
Hemoglobinopathies

Answer 133

Pappenheimer bodies (Wright’s stain)
Siderotic granules (Prussian blue)

Question 134

Multiple, uniform, evenly distributed dark blue granules

Answer 134

Basophilic stippling

Question 135

RNA

Answer 135

Basophilic stippling

Question 136

▪ Megaloblastic anemia
▪ Thalassemia
▪ Hemoglobinopathies
▪ Alcoholism
▪ Pyrimidine-5nucleotidase deficiency

Answer 136

Basophilic stippling Fine

Question 137

▪ Lead poisoning

Answer 137

Basophilic stippling Coarse

Question 138

Single or multiple purplish inclusions on the RBC periphery

Answer 138

Heinz bodies

Question 139

Supravital stains: CV, BCB

Answer 139

Heinz bodies

Question 140

Precipitated Hb

Answer 140

Heinz bodies

Question 141

G6PD deficiency (favism)
Naphthalene ball ingestion
Hemoglobinopathies
Thalassemia major
Sulfonamides
Hb Koln and Hb Zurich
Post-splenectomy

Answer 141

Heinz bodies

Question 142

Small-greenish blue

Answer 142

Hb H inclusions

Question 143

“Pitted gold ball appearance”

Answer 143

Hb H inclusions

Question 144

Supravital stain: BCB

Answer 144

Hb H inclusions

Question 145

Fingerlike or quartz like crystal of dense Hb protruding from the RBC membrane

Answer 145

Hb SC crystal

Question 146

Hexagonal crystal of dense Hb

Answer 146

Hb C crystal

Question 147

Also known as hemozoin/hematin

Answer 147

Malarial inclusions

Question 148

Plasmodium spp.

Answer 148

Malarial inclusions

Question 149

Malaria

Answer 149

Malarial inclusions

Question 150

Resembles maltese cross/tetrads

Answer 150

Babesia inclusion

Question 151

Babesia spp.

Answer 151

Babesia inclusion

Question 152

Babesiosis/Piroplasmosis

Answer 152

Babesia inclusion

Question 153

− Macrophage-mediated hemolysis

Answer 153

Extravascular hemolysis

Question 154

− Occurs in the spleen

Answer 154

Extravascular hemolysis

Question 155

− 90% of RBCs are destroyed

Answer 155

Extravascular hemolysis

Question 156

− Mechanical hemolysis

Answer 156

Intravascular hemolysis

Question 157

− Occurs within the lumen of blood vessels

Answer 157

Intravascular hemolysis

Question 158

− Extremely damaged cells are being destroyed within the circulation before they reach the liver or the spleen

Answer 158

Intravascular hemolysis

Question 159

− 10% of RBCs are destroyed

Answer 159

Intravascular hemolysis

Question 160

− Removal of senescent and damaged red blood cells in the spleen

Answer 160

▪ Splenic culling

Question 161

▪− Removal of RBC inclusion bodies in the spleen

Answer 161

Splenic pitting

Question 162

NOTE: when red blood cells are destroyed, only the [?]are recycled for reuse

Answer 162

iron and globin chains

Question 163

▪ Function: to transport oxygen to the tissue and carbon dioxide from tissues to the lungs

Answer 163

Hemoglobin

Question 164

Hemoglobin Composition:

Answer 164

4 heme
4 globin

Question 165

– consists of protoporphyrin IX and ferrous iron

Answer 165

4 heme

Question 166

– consists of 2 identical pairs of unlike polypeptide chains

Answer 166

4 globin

Question 167

✓ every heme group is capable of carrying [?] of oxygen, therefore each Hb molecule is able to transport [?] of oxygen

Answer 167

1 mole

4 moles

Question 168

✓ 1 gram Hb = can carry [?] of oxygen

Answer 168

1.34 mL

Question 169

✓ 1 gram Hb = can carry [?] of iron

Answer 169

3.47 mg

Question 170

✓ there are 4 pyrrole rings for every [?] of iron

Answer 170

1 molecule

Question 171

: occurs in the mitochondria

Answer 171

▪ Heme synthesis

Question 172

: occurs in the ribosomes

Answer 172

▪ Globin synthesis

Question 173

Alpha

Answer 173

141

Question 174

Zeta
Beta
Gamma
Delta
Epsilon

Answer 174

146

Question 175

Alpha
Zeta

Answer 175

Ch 16

Question 176

Beta
Gamma
Delta
Epsilon

Answer 176

Ch 11

Question 177

2 alpha, 2 beta

Answer 177

A1

Question 178

> 95%

Answer 178

A1

Question 179

Predominant Hb among the adults

Answer 179

A1

Question 180

2 alpha, 2 delta

Answer 180

A2

Question 181

<3%

Answer 181

A2

Question 182

2 alpha, 2 gamma

Answer 182

F

Question 183

1-2%

Answer 183

F

Question 184

Predominant Hb during the hepatic phase

Answer 184

F

Question 185

Major Hb of newborn

Answer 185

F

Question 186

Embryonal hemoglobin

Question 187

2 zeta, 2 epsilon

Answer 187

Gower I

Question 188

2 alpha, 2 epsilon

Answer 188

Gower II

Question 189

2 zeta, 2 gamma

Answer 189

Portland

Question 190

Cherry red

Answer 190

Carboxyhemoglobin

Question 191

Chocolate brown

Answer 191

Methemoglobin

Question 192

Mauve lavender

Answer 192

Sulfhemoglobin

Question 193

Reversible

Answer 193

Carboxyhemoglobin Methemoglobin

Question 194

Not Reversible

Answer 194

Sulfhemoglobin

Question 195

Hb bounded with CO

Answer 195

Carboxyhemoglobin

Question 196

Hb that contains iron in oxidized or ferric state (Fe3+)

Answer 196

Methemoglobin

Question 197

Hb bounded with sulfur

Answer 197

Sulfhemoglobin

Question 198

The affinity of hemoglobin to CO is 200 times greater than oxygen

Answer 198

Carboxyhemoglobin

Question 199

Cannot transport oxygen

Answer 199

Methemoglobin

Question 200

Once formed, it will stay in the RBC during its entire 120day lifespan

Answer 200

Sulfhemoglobin

Question 201

Associated with C. perfringens infection

Answer 201

Sulfhemoglobin

Question 202

Formation is caused by oxidizing drugs (acetanilid, phenacetin, and sulfonamides)

Answer 202

Sulfhemoglobin

Question 203

▪ Graphically describe the relationship between oxygen content and partial pressure of oxygen

Answer 203

Oxygen Dissociation Curve

Question 204

▪ Shape of the curve:

Answer 204

sigmoid (affected by 2,3-DPG and oxygen)

Question 205

(Hb won’t let go its oxygen)

Answer 205

Increase in oxygen affinity – hemoglobin has increased affinity for oxygen

Question 206

(Hb releases oxygen)

Answer 206

Decrease in oxygen affinity – hemoglobin has decreased affinity for oxygen

Question 207

– increased oxygen affinity

Answer 207

▪ Shift to the left

Question 208

– decreased oxygen affinity

Answer 208

▪ Shift to the right

Question 209

– hemoglobin’s affinity for oxygen is influenced by pH

Answer 209

▪ Bohr effect

Question 210

– hemoglobin’s affinity for oxygen is influenced by carbon dioxide

Answer 210

▪ Haldane effect

Question 211

Carbon Dioxide
Increased:
Decreased:

Answer 211

shift to the R ; shift to the L

Question 212

pH
Increased:
Decreased:

Answer 212

shift to the L ; shift to the R

Question 213

2,3-DPG
Increased:
Decreased:

Answer 213

shift to the R ; shift to the L

Question 214

Exercise
Increased:

Answer 214

shift to the R (due to lactic acid)

Question 215

Temperature
Increased:
Decreased:

Answer 215

shift to the R ; shift to the L

Question 216

Hb F:

Answer 216

shift to the L

Question 217

Hb Chesapeake:

Answer 217

shift to the L

Question 218

Hb Kansas:

Answer 218

shift to the R

Question 219

▪ Together with protoporphrin IX, [?] is important for the synthesis of heme

Answer 219

iron

Question 220

– functional, absorbable form in the intestine

Answer 220

▪ Ferrous iron

Question 221

▪ Normal adult iron level – approximately [?] (60% in the circulation; 40% in the storage form)

Answer 221

400 mg

Question 222

▪ Storage form of iron
o – short-term storage form of iron
o – long-term storage form of iron

Answer 222

Ferritin

Hemosiderin

Question 223

o Also known as siderophilin

Answer 223

▪ Transferrin

Question 224

o Transport protein of iron

Answer 224

▪ Transferrin

Question 225

o Produced by the liver

Answer 225

▪ Hepcidin

Question 226

o Negative regulator of intestinal iron absorption

Answer 226

▪ Hepcidin

Question 227

o Suppresses the release of iron from macrophage

Answer 227

▪ Hepcidin

Question 228

o Plays an important role in anemia of chronic inflammation

Answer 228

▪ Hepcidin

Question 229

▪ Storage site of iron in the body

Answer 229

o Liver (major) o Bone marrow o Spleen

Question 230

▪ Main function: primary defense against foreign invaders such as bacteria, viruses and other foreign antigens

Answer 230

White blood cells

Question 231

White blood cells Compartments in the body:

Answer 231

bone marrow, peripheral blood, tissues

Question 232

: most valuable and reliable criterion for deciding whether a cell is mature or immature

Answer 232

Nuclear chromatin pattern

Question 233

According to granularity

Question 234

According to segmentation

Question 235

According to function

Question 236

Granulocytes

Answer 236

Neutrophil Basophil Eosinophil

Question 237

Agranulocytes

Answer 237

Monocyte Lymphocyte

Question 238

Polymorphonuclear cells

Answer 238

Neutrophil Basophil Eosinophil

Question 239

Mononuclear cells

Answer 239

Monocyte Lymphocyte

Question 240

Phagocytes

Answer 240

Neutrophil Monocyte Eosinophil

Question 241

Immunocytes

Answer 241

Lymphocyte

Question 242

▪ Earliest recognizable blast

Answer 242

Myeloblast

Question 243

▪ No visible granules

Answer 243

Myeloblast

Question 244

▪ The nucleus is made up of a smooth, delicate, uniformly distributed chromatin pattern (lacy chromatin pattern)

Answer 244

Myeloblast

Question 245

▪ First appearance of primary granules (azurophilic/non-specific granules)

Answer 245

Promyelocyte

Question 246

▪ Appearance of secondary or specific granules

Answer 246

Myelocyte

Question 247

▪ Dawn of neutrophilia

Answer 247

Myelocyte

Question 248

▪ Also known as Juvenile cells

Answer 248

Metamyelocyte

Question 249

▪ Appearance of tertiary granules

Answer 249

Metamyelocyte

Question 250

▪ Nucleus: kidney bean or peanut shaped

Answer 250

Metamyelocyte

Question 251

▪ Indentation of the nucleus: <50% of the width of the nucleus

Answer 251

Metamyelocyte

Question 252

▪ Also known as Stab cell/Staff cell

Answer 252

Band cell

Question 253

▪ Appearance of secretory granules

Answer 253

Band cell

Question 254

▪ Nucleus: sausage-shaped

Answer 254

Band cell

Question 255

▪ Indentation of the nucleus: >50% of the width of the nucleus

Answer 255

Band cell

Question 256

▪ First immature WBC to be released in the circulation

Answer 256

Band cell

Question 257

▪ Youngest cell in the granulocytic series to normally appear in the peripheral blood

Answer 257

Band cell

Question 258

▪ Most common WBC in normal peripheral blood

Answer 258

Neutrophil

Question 259

Neutrophil Two forms in the peripheral blood:

Answer 259

Segmenters and Bands

Question 260

Neutrophil Lifespan:

Answer 260

o 9-10 days (Steininger)
o 5 days (Brown)

Question 261

o Tissue neutrophil

Answer 261

Ferrata cell

Question 262

o Associated with subacute bacterial endocarditis

Answer 262

Ferrata cell

Question 263

: o Described as drumstick

Answer 263

▪ Barr body

Question 264

o Described as drumstick

Answer 264

▪ Barr body

Question 265

o Represents the second X chromosome in females and may be seen in 2-3% of the neutrophils in females

Answer 265

▪ Barr body

Question 266

Pools of neutrophils in the Bone marrow:

Answer 266

 Mitotic/Proliferating pool
 Storage/Maturation pool

Question 267

− Myeloblasts, Promyelocytes, Myelocytes

Answer 267

 Mitotic/Proliferating pool

Question 268

− Cells undergoing cell division

Answer 268

 Mitotic/Proliferating pool

Question 269

− Metamyelocytes, Bands, Segmented neutrophils

Answer 269

 Storage/Maturation pool

Question 270

− Cells no longer undergoing cell division but progressively maturing

Answer 270

 Storage/Maturation pool

Question 271

o Pools of neutrophils in the circulation:

Answer 271

 Circulating pool (50%)
 Marginating pool (50%)

Question 272

Azurophilic/nonspecific granules

Answer 272

Primary granules

Question 273

Produced by Promyelocytes

Answer 273

Primary granules

Question 274

Primary granules:

Answer 274

▪ MPO
▪ Cathepsins
▪ Acid beta-glycerophosphatase
▪ Defensins
▪ Elastase
▪ Proteinase-3

Question 275

Specific granules

Answer 275

Secondary granules

Question 276

Produced by Myelocyte

Answer 276

Secondary granules

Question 277

Secondary granules:

Answer 277

▪ Lactoferrin
▪ Collagenase
▪ Gelatinase
▪ beta-2 microglobulin
▪ Lipocalin

Question 278

Produced by metamyelocyte

Answer 278

Tertiary granules

Question 279

▪ Gelatinase
▪ Collagenase
▪ Lysozyme
▪ Acetyltransferase
▪ Beta-2 microglobulin

Answer 279

Tertiary granules

Question 280

Also known as secretory vesicles

Answer 280

Secretory granules

Question 281

Produced by Band cell

Answer 281

Secretory granules

Question 282

Secretory granules:

Answer 282

▪ ALP
▪ Vesicle-associated membrane-2
▪ CD13, CD10, CD14, CD16, CD18

Question 283

▪ Plays a major role in defense against parasitic invasion and in hypersensitivity reaction

Answer 283

Eosinophil

Question 284

▪ Eosinophil Important products:

Answer 284

MBP and Charcot-Leyden crystals

Question 285

– an arginine-rich protein that plays an important role in the eosinophil’s ability to damage parasites

Answer 285

▪ Major Basic Protein

Question 286

: − Hexagonal pyramidal crystals

Answer 286

▪ Charcot-Leyden crystals

Question 287

− Found in the nasal mucus of patients with allergic asthma, pleural fluid of patients w/ pulmonary eosinophilic infiltrates, and stool of patients w/ parasitic infections

Answer 287

▪ Charcot-Leyden crystals

Question 288

▪ Mediator of Immediate hypersensitivity reaction

Answer 288

Basophil

Question 289

cells have specific receptor for IgE

Answer 289

▪ Basophils and mast

Question 290

▪ Basophils Important products:

Answer 290

Histamine and Heparin

Question 291

Main function: phagocytosis

Answer 291

▪ Monocyte

Question 292

Slightly immature cells whose ultimate goal is to enter the tissues and mature into macrophages

Answer 292

▪ Monocyte

Question 293

Size: 15-20 um

Answer 293

▪ Monocyte

Question 294

o Chromatin pattern: lacelike

Answer 294

Monocyte

Question 295

o Nucleus: horseshoe or tulip shape

Answer 295

Monocyte

Question 296

o Cytoplasm: blue-gray with fine azure granules often referred to as azure dust or a ground glass appearance

Answer 296

Monocyte

Question 297

▪ Monocyte Maturation series:

Answer 297

monoblast → promonocyte → monocyte → macrophage

Question 298

o There is no storage pool in the bone marrow

Answer 298

Monocyte

Question 299

o Proliferation in the bone marrow: 55 hours

Answer 299

Monocyte

Question 300

spends about 12 hours in the peripheral blood before going to the tissues

Answer 300

Monocyte

Question 301

o The marginal pool in the peripheral blood is 3.5 times greater than the circulating pool

Answer 301

Monocyte

Question 302

▪ Main function: immune response

Answer 302

Lymphocyte

Question 303

▪ Serves as a “marker cell” for estimating the size of surrounding cells

Answer 303

Lymphocyte

Question 304

– most small lymphocytes

Answer 304

▪ T-lymphocytes

Question 305

– most large lymphocytes

Answer 305

▪ B-lymphocytes

Question 306

– third population of lymphocytes

Answer 306

▪ NK cells

Question 307

▪ Lifespan: several months to years

Answer 307

Lymphocyte

Question 308

▪ Mononuclear cells with round or oval and smooth or irregular margins

Answer 308

Plasma cells

Question 309

▪ The eccentrically located nucleus is composed of blocks of heterochromatin resembling a tortoise shell

Answer 309

Plasma cells

Question 310

▪ Nucleus exhibits a cartwheel pattern

Answer 310

Plasma cells

Question 311

▪ The area next to the nucleus containing the Golgi apparatus is unstained (Hof)

Answer 311

Plasma cells

Question 312

: located on the cytoplasm that may contain round, discrete globules that appear paleclue, or occasionally red which contains immunoglobulins

Answer 312

▪ Russel bodies

Question 313

– cluster of Russel bodies

Answer 313

▪ Morula cell/grape cell/Mott cells

Question 314

 Associated with increased RBC count, hemoglobin, hematocrit

Answer 314

Polycythemia

Question 315

 May be classified as relative or absolute

Answer 315

Polycythemia

Question 316

NOTE: A hematocrit value of [?] in men and [?] in
women is often diagnostic of Polycythemia

Answer 316

> 52%

> 50%

Question 317

Refers to true increase in red cell mass

Answer 317

ABSOLUTE POLYCYTHEMIA

Question 318

– due to bone marrow defect

Answer 318

▪ Primary Polycythemia

Question 319

Refers to true increase in red cell mass

Answer 319

ABSOLUTE POLYCYTHEMIA

Question 320

– due to kidney defect

Answer 320

▪ Secondary Polycythemia

Question 321

▪ Chronic myeloproliferative disorder

Answer 321

Absolute Primary Polycythemia

Question 322

▪ Due to mutation of JAK2 gene

Answer 322

Absolute Primary Polycythemia

Question 323

▪ Pancytosis: absolute increased in RBC, WBC, platelets Absolute

Answer 323

Absolute Primary Polycythemia

Question 324

▪ Panhyperplasia: the bone marrow is hypercellular showing an overall increase

Answer 324

Absolute Primary Polycythemia

Question 325

Characterized by hyperviscous blood (due to increased RBCs)

Answer 325

Absolute Primary Polycythemia

Question 326

▪ Prone to IDA (therapeutic phlebotomy)

Answer 326

Absolute Primary Polycythemia

Question 327

▪ Hallmark of PV:

Answer 327

plethora

Question 328

Absolute Primary Polycythemia Clinical features:

Answer 328

▪ ESR is decreased
▪ EPO is decreased
▪ LAP is increased
▪ Dacryocyte in PBS is a common finding

Question 329

▪ Due to increased level of EPO

Answer 329

Absolute Secondary Polycythemia

Question 330

▪ Residence at high altitudes

Answer 330

Absolute Secondary Polycythemia

Question 331

▪ Chronic pulmonary disease
▪

Answer 331

Absolute Secondary Polycythemia

Question 332

CHF

Answer 332

Absolute Secondary Polycythemia

Question 333

▪ Heavy smoking

Answer 333

Absolute Secondary Polycythemia

Question 334

▪ Methemoglobinemia

Answer 334

Absolute Secondary Polycythemia

Question 335

▪ Tumor of the kidneys

Answer 335

Absolute Secondary Polycythemia

Question 336

▪ Due to decrease in the fluid (plasma) portion of the blood that gives the appearance of an increased red cell mass in relation to total blood volume rather than a true increase in red cell mass

Answer 336

RELATIVE POLYCYTHEMIA

Question 337

▪ NOT a hematologic disorder

Answer 337

RELATIVE POLYCYTHEMIA

Question 338

▪ Actual number of RBC in the blood is not increased, but the number of cells per unit volume of blood is increased

Answer 338

RELATIVE POLYCYTHEMIA

Question 339

1. Dehydration secondary to diarrhea, vomiting, excessive sweating, burns, anaphylaxis, and diuretics

Answer 339

RELATIVE POLYCYTHEMIA

Question 340

2. Anxiety and stress

Answer 340

RELATIVE POLYCYTHEMIA

Question 341

3. Tobacco smoking

Answer 341

RELATIVE POLYCYTHEMIA

Question 342

4. Gaisbock’s syndrome

Answer 342

RELATIVE POLYCYTHEMIA

Question 343

− also known as Spurious polycythemia or Stress syndrome

Answer 343

Gaisbock’s syndrome

Question 344

− Associated with smoking, CVD, hypertension, and diuretic therapy

Answer 344

Gaisbock’s syndrome

Question 345

 A decrease in red blood cells, hemoglobin, and hematocrit below the reference range for healthy individuals of the same age, sex, and race, under similar environmental conditions

Answer 345

Anemia

Question 346

Mechanisms of Anemia

Answer 346

A. Due to Production B. Due to Destruction

Question 347

A. Due to Production

Answer 347

▪ Ineffective erythropoiesis
▪ Insufficient erythropoiesis

Question 348

B. Due to Destruction

Answer 348

▪ Intrinsic defects in the RBC membrane, enzyme, or hemoglobin
▪ Extrinsic causes such as antibody-mediated process, mechanical fragmentation, or infectionrelated

Question 349

Test for Accelerated RBC destruction

Answer 349

1. Lactate dehydrogenase
2. Indirect bilirubin
3. Chromium Radioisotope: the reference method for RBC survival studies by ICSH

Question 350

Laboratory Diagnosis of Anemia

Answer 350

1. CBC and RBC indices
2. Reticulocyte count
3. Peripheral Blood Smear Examination
4. Bone Marrow Examination
5. H/H

Question 351

– serves as an important tool to assess the bone marrow’s ability to increase RBC production in response to anemia

Answer 351

2. Reticulocyte count

Question 352

– indicated for a patient with an unexplained anemia, fever of unknown origin, or suspected hematologic malignancy

Answer 352

4. Bone Marrow Examination

Question 353

– widely used tests for anemia

Answer 353

5. H/H

Question 354

▪ A condition in which there is a peripheral blood pancytopenia

Answer 354

Aplastic Anemia

Question 355

▪ Pancytopenia: dec RBC, WBC, Platelets, Reticulocytes

Answer 355

Aplastic Anemia

Question 356

▪ Lymphocytes are the predominant cell in the peripheral blood (less affected)

Answer 356

Aplastic Anemia

Question 357

Clinical Features:
▪ Bleeding
▪ Infection
▪ Anemia
▪ No splenomegaly
▪ No lymphadenopathy

Answer 357

Aplastic Anemia

Question 358

▪ Genetic defect

Answer 358

Aplastic Anemia

Question 359

▪ Ionizing radiation

Answer 359

Aplastic Anemia

Question 360

▪ Chemicals

Answer 360

Aplastic Anemia

Question 361

▪ Parvovirus B19

Answer 361

Aplastic Anemia

Question 362

▪ Benzene

Answer 362

Aplastic Anemia

Question 363

▪ Chloramphenicol (most common cause)

Answer 363

Aplastic Anemia

Question 364

▪ Trinitrotoluene

Answer 364

Aplastic Anemia

Question 365

▪ Arsenic

Answer 365

Aplastic Anemia

Question 366

▪ Fanconi Anemia

Answer 366

Hereditary Aplastic Anemia

Question 367

▪ Diamond-Blackfan anemia

Answer 367

Hereditary Aplastic Anemia

Question 368

▪ Chronic Kidney Disease

Answer 368

Acquired Aplastic Anemia

Question 369

▪ Myelophthisic anemia

Answer 369

Acquired Aplastic Anemia

Question 370

▪ Also known as Congenital Aplastic Anemia

Answer 370

Fanconi Anemia

Question 371

▪ Autosomal recessive

Answer 371

Fanconi Anemia

Question 372

▪ Pancytopenia

Answer 372

Fanconi Anemia

Question 373

▪ Normocytic anemia

Answer 373

Fanconi Anemia

Question 374

▪ Low birth weight (<2,500 gram)

Answer 374

Fanconi Anemia

Question 375

▪ Skin hyperpigmentation (café au lait spots)

Answer 375

Fanconi Anemia

Question 376

▪ Short stature

Answer 376

Fanconi Anemia

Question 377

▪ Renal malformations

Answer 377

Fanconi Anemia

Question 378

▪ Microcephaly

Answer 378

Fanconi Anemia

Question 379

▪ Mental retardation

Answer 379

Fanconi Anemia

Question 380

▪ Hypogonadism

Answer 380

Fanconi Anemia

Question 381

▪ Strabismus

Answer 381

Fanconi Anemia

Question 382

▪ Also known as Congenital Pure Red Cell Aplasia

Answer 382

Diamond-Blackfan Anemia

Question 383

▪ Defective/reduced CFU-E

Answer 383

Diamond-Blackfan Anemia

Question 384

▪ Caused by a mutation in RPS19 gene; idiopathic (Steininger)

Answer 384

Diamond-Blackfan Anemia

Question 385

▪ Normocytic anemia w/ normal leukocyte and platelet count and a marked decrease in marrow erythroblasts

Answer 385

Diamond-Blackfan Anemia

Question 386

▪ Also known as leucoerythroblastic anemia

Answer 386

Myelophthisic Anemia

Question 387

▪ Common finding in patients with carcinoma

Answer 387

Myelophthisic Anemia

Question 388

▪ Results when the bone marrow is replaced by abnormal cells such as metastatic tumor cells, leukemic cells, fibroblasts, and inflammatory cells (found in miliary TB and fungal infections)

Answer 388

Myelophthisic Anemia

Question 389

= invasion of abnormal cell

Answer 389

▪ Myelophthisis

Question 390

Myelophthisic Anemia Lab Picture:

Answer 390

▪ Normocytic anemia

Question 391

▪ Normocytic anemia
▪  Reticulocyte, Teardrop cells, nRBCs, immature myeloid cells in the peripheral blood, presence of abnormal cells in the bone marrow Anemia of Chronic Kidney Disease
▪ Anemia is due to inadequate production of EPO by the kidneys
▪  EPO, presence of Burr cells (Uremia)

Answer 391

Myelophthisic Anemia

Question 392

▪ dec Reticulocyte, Teardrop cells, nRBCs, immature myeloid cells in the peripheral blood, presence of abnormal cells in the bone marrow

Answer 392

Myelophthisic Anemia

Question 393

▪ Anemia is due to inadequate production of EPO by the kidneys

Answer 393

Anemia of Chronic Kidney Disease

Question 394

▪ dec EPO, presence of Burr cells (Uremia)

Answer 394

Anemia of Chronic Kidney Disease

Question 395

▪ Impaired DNA synthesis affects all rapidly dividing cells of the body, including the skin, GIT, and bone marrow

Answer 395

ANEMIA OF ABNORMAL NUCLEAR DEVELOPMENT

Question 396

▪ Vitamin B12 and Folate are essential in DNA synthesis

Answer 396

ANEMIA OF ABNORMAL NUCLEAR DEVELOPMENT

Question 397

▪ Deficiencies of either vitamin impair DNA replication, halt cell division, and increase apoptosis, which results in ineffective erythropoiesis and megaloblastic morphology

Answer 397

ANEMIA OF ABNORMAL NUCLEAR DEVELOPMENT

Question 398

▪ Dietary source: meat

Answer 398

Vitamin B12 Deficiency

Question 399

▪ The liver stores adequate amount of Vitamin B12 for several years if no more is ingested

Answer 399

Vitamin B12 Deficiency

Question 400

: forms a protective complex with Vitamin B12 that is transported down the GIT

Answer 400

▪ Intrinsic factor

Question 401

▪ Vitamin B12 is maximally absorbed in the

Answer 401

ileum

Question 402

Vitamin B12 Deficiency Causes:

Answer 402

▪ Inadequate intake
▪ Increased need
▪ Impaired absorption

Question 403

▪ Autoimmune disorder characterized by impaired absorption of vitamin B12 due to lack of IF

Answer 403

Pernicious Anemia

Question 404

▪ Most common form of Vitamin B12 deficiency

Answer 404

Pernicious Anemia

Question 405

▪ More common in people with blood type A

Answer 405

Pernicious Anemia

Question 406

▪ The organism has the ability to split vitamin B12 from IF, rendering the vitamin unavailable for host absorption

Answer 406

D. latum infection

Question 407

▪ Portions of the intestines becomes stenotic as a result of surgery or inflammation

Answer 407

Blind loop syndrome

Question 408

▪ These sites can become overgrown with intestinal bacteria that compete effectively with the host for available vitamin B12

Answer 408

Blind loop syndrome

Question 409

▪ Causes Vitamin B12 malabsorption

Answer 409

Imerslund-Grasbeck syndrome

Question 410

▪ Not related to IF deficiency/defect

Answer 410

Imerslund-Grasbeck syndrome

Question 411

▪ Defect in cubilin/amnionless receptor (Henry’s)

Answer 411

Imerslund-Grasbeck syndrome

Question 412

▪ Provides a measure of body’s ability to secrete viable IF and absorb orally administered 57Co-labeled B12 in the ileum

Answer 412

Schilling Test (Classical Test)

Question 413

Schilling Test (Classical Test) Specimen requirement

Answer 413

▪ Fasting specimen
▪ A 24-hour urine collection is begun immediately upon administration of the labeled B12 by mouth Interpretation

Question 414

Schilling Test (Classical Test)
Phase 1 (radiolabeled B12 w/o IF)
▪ >7% of labeled B12 is excreted =
▪ <7% of labeled B12 is excreted =

Answer 414

Dietary B12 deficiency

Proceed to Phase 2

Question 415

Schilling Test (Classical Test)
Phase 2 (radiolabeled B12 w/IF)
▪ >7% of labeled B12 is excreted =
▪ <7% of labeled B12 is excreted =

Answer 415

Pernicious anemia

Malabsorption syndrome (Tropical Sprue, D. latum, Blind Loop syndrome)

Question 416

Sources of Folate:

Answer 416

green leafy vegetables

Question 417

▪ Inadequate intake

Answer 417

Folate Deficiency

Question 418

▪ Increased need

Answer 418

Folate Deficiency

Question 419

▪ Impaired absorption

Answer 419

Folate Deficiency

Question 420

▪ Excessive loss due to renal dialysis

Answer 420

Folate Deficiency

Question 421

▪ Alcohol (alcohol interferes with folate metabolism)

Answer 421

Folate Deficiency

Question 422

▪ Lacks hypersegmented neutrophil and oval macrocytes in the peripheral blood and megaloblasts in the bone marrow

Answer 422

Macrocytic Non-Megaloblastic Anemia

Question 423

Macrocytic Non-Megaloblastic Anemia ▪ Physiologic cause:

Answer 423

newborn

Question 424

Macrocytic Non-Megaloblastic Anemia ▪ Pathologic cause:

Answer 424

liver disease, chronic alcoholism, bone marrow failure

Question 425

CBC Folate deficiency Vitamin B12 deficiency

Pancytopenia ([?]RBC, WBC, Platelet count)
[?] MCV, MCH
[?] RDW
[?] Reticulocyte count

Answer 425

Dec
Inc
Inc
Dec

Question 426

PBS examination Folate deficiency Vitamin B12 deficiency:

Answer 426

Macroovalocytosis
Teardrop cells
nRBCs
Hypersegmented neutrophils

Question 427

Bone marrow examination Folate deficiency Vitamin B12 deficiency:

Answer 427

Presence of megaloblasts
Inc M:E ratio
Hypercellular bone marrow

Question 428

(+) anti-parietal cells, anti-IF

Answer 428

Vitamin B12 deficiency

Question 429

Achlorhydria

Answer 429

Vitamin B12 deficiency

Question 430

(+) D. latum eggs

Answer 430

Vitamin B12 deficiency

Question 431

Gastric analysis NORMAL

Answer 431

Folate deficiency

Question 432

Anemia of Iron and Heme Metabolism Mechanisms:
1. Deficiency of[?]
2. Defective release of[?] from macrophages (Anemia of Chronic Inflammation)
3. Defective utilization of[?] within the erythroblast (SDA, lead poisoning)

Answer 432

raw material (e.g., iron)

stored iron

iron

Question 433

Tests used to differentiate iron metabolism disorders:

Answer 433

1. Serum Ferritin
2. Serum Iron
3. FEP
4. TIBC
5. Transferrin saturation
6. Zinc Erythrocyte Porphyrin

Question 434

▪ Reflects the body’s tissue iron stores

Answer 434

Serum Ferritin

Question 435

▪ A good indicator of iron storage status

Answer 435

Serum Ferritin

Question 436

▪ First laboratory test to become abnormal when iron stores begin to decrease

Answer 436

Serum Ferritin

Question 437

▪ Decreased only in IDA

Answer 437

Serum Ferritin

Question 438

▪ Helpful in cases where the diagnosis is not obvious from other laboratory tests

Answer 438

Serum Iron

Question 439

▪ Normally, red cells produced slightly more protoporphyrin than is needed

Answer 439

FEP

Question 440

▪ However, when iron is deficient, protoporphyrin levels builds up in RBCs than the normal level

Answer 440

FEP

Question 441

▪ Indirect measurement of transferrin concentration

Answer 441

TIBC

Question 442

▪ Measures the binding site

Answer 442

TIBC

Question 443

▪ (dec Anemia of chronic inflammation; inc IDA)

Answer 443

TIBC

Question 444

▪ Obtained through the measurements of serum iron and TIBC

Answer 444

Transferrin saturation

Question 445

%Transferrin saturation =

Answer 445

serum iron/TIBC x 100

Question 446

▪ Measures unused protoporphyrin

Answer 446

Zinc Erythrocyte Porphyrin Test

Question 447

▪ Increased in IDA, Lead poisoning, and Porphyria

Answer 447

Zinc Erythrocyte Porphyrin Test

Question 448

▪ Most common form of anemia

Answer 448

Iron Deficiency Anemia

Question 449

▪ The individual may not exhibit signs or symptoms until the appearance of Frank anemia

Answer 449

Iron Deficiency Anemia

Question 450

▪ Microcytic, hypochromic anemia

Answer 450

Iron Deficiency Anemia

Question 451

Iron Deficiency Anemia
▪ [?] Iron
▪ [?] TIBC and FEP
▪ Normal Reticulocyte count
▪ [?] Reticulocytes after iron therapy

Answer 451

Dec
Inc
Inc

Question 452

Iron Deficiency Anemia Causes

Answer 452

Inadequate intake
Increased body demand (pregnancy, growing children)
Impaired absorption
Chronic blood loss due to infection (Hookworm infection)
Marching anemia: develops when RBCs are hemolyzed by footpounding trauma and iron is lost (hemoglobinuria is a common finding)

Question 453

Chronic blood loss due to infection (?)

Answer 453

Hookworm infection

Question 454

Chronic blood loss due to infection (?)

Answer 454

Hookworm infection

Question 455

: develops when RBCs are hemolyzed by footpounding trauma and iron is lost (hemoglobinuria is a common finding)

Answer 455

Marching anemia

Question 456

Stages of iron loss

Answer 456

▪ Stage 1 – progressive loss of storage iron
▪ Stage 2 – exhaustion of the storage pool of iron
▪ Stage 3 – Frank anemia (storage pool and circulatory iron is depleted)

Question 457

Stages of iron loss

Answer 457

▪ Stage 1 – progressive loss of storage iron
▪ Stage 2 – exhaustion of the storage pool of iron
▪ Stage 3 – Frank anemia (storage pool and circulatory iron is depleted)

Question 458

– progressive loss of storage iron

Answer 458

▪ Stage 1

Question 459

– exhaustion of the storage pool of iron

Answer 459

▪ Stage 2

Question 460

– exhaustion of the storage pool of iron

Answer 460

▪ Stage 2

Question 461

– Frank anemia (storage pool and circulatory iron is depleted)

Answer 461

▪ Stage 3

Question 462

▪ Glossitis

Answer 462

Iron Deficiency Anemia

Question 463

▪ Glossitis

Answer 463

Iron Deficiency Anemia

Question 464

▪ Angular cheilosis

Answer 464

Iron Deficiency Anemia

Question 465

– inflamed cracks at the corner of the mouth

Answer 465

Angular cheilosis

Question 466

– inflamed cracks at the corner of the mouth

Answer 466

Angular cheilosis

Question 467

▪ Koilonychia

Answer 467

Iron Deficiency Anemia

Question 468

▪ Koilonychia

Answer 468

Iron Deficiency Anemia

Question 469

– spooning of the fingernails

Answer 469

Koilonychia

Question 470

▪ Pica

Answer 470

Iron Deficiency Anemia

Question 471

▪ Pagophagia

Answer 471

Iron Deficiency Anemia

Question 472

▪ Decreased: RBC count, H/H, MCV, MCH, MCHC

Answer 472

Iron Deficiency Anemia

Question 473

▪ Increased RDW (anisocytosis)

Answer 473

Iron Deficiency Anemia

Question 474

▪ Increased RDW (anisocytosis)

Answer 474

Iron Deficiency Anemia

Question 475

are iron-containing normoblasts found in a normal bone marrow

Answer 475

Sideroblastic Anemia ▪ Sideroblasts

Question 476

▪ The iron deposits are identified using Prussian blue stain, and the resulting abnormal cells are identified as ringed sideroblasts

Answer 476

Sideroblastic Anemia

Question 477

▪ Caused by: defective iron loading (accumulation of erythroid precursor in the mitochondria) due to deficiency of ALA synthetase

Answer 477

Sideroblastic Anemia

Question 478

Sideroblastic Anemia Hereditary:

Answer 478

▪ Hereditary Sideroblastic Anemia

Question 479

Sideroblastic Anemia Acquired:

Answer 479

▪ Refractory Anemia w/ Ringed Sideroblasts
▪ Idiopathic Acquired Sideroblastic Anemia
▪ Primary Idiopathic Sideroblastic Anemia
▪ Secondary Sideroblastic Anemia

Question 480

▪ Severe anemia (Hct = <20%)

Answer 480

Hereditary Sideroblastic Anemia

Question 481

▪ Dimorphic population of RBCs:
✓ Normocytic, normochromic
✓ Microcytic, hypochromic

Answer 481

Hereditary Sideroblastic Anemia

Question 482

▪ (+) Target cells and basophilic stippling

Answer 482

Hereditary Sideroblastic Anemia

Question 483

▪ inc iron and % transferrin saturation

Answer 483

Hereditary Sideroblastic Anemia

Question 484

▪ More common SDA

Answer 484

Primary Idiopathic SDA

Question 485

▪ Moderate anemia (Hct = 25-30%)

Answer 485

Primary Idiopathic SDA

Question 486

▪ (+) normocytes and macrocytes w/ few microcytes

Answer 486

Primary Idiopathic SDA

Question 487

▪ Erythroid hyperplasia w/ ringed sideroblasts in all stages of development

Answer 487

Primary Idiopathic SDA

Question 488

▪ Due to toxins and drugs that interfere w/ heme synthesis

Answer 488

Secondary SDA

Question 489

▪ Alcoholism, lead poisoning, TB drugs, and chloramphenicol

Answer 489

Secondary SDA

Question 490

▪ Second most common anemia

Answer 490

Anemia of Chronic Inflammation

Question 491

▪ Associated with infections, inflammatory, or malignant diseases of more than 1 or 2 months of duration

Answer 491

Anemia of Chronic Inflammation

Question 492

▪ Most common anemia among hospitalized patients

Answer 492

Anemia of Chronic Inflammation

Question 493

▪ Iron appears to be trapped in macrophages; therefore iron is not made available for reutilization in normoblasts

Answer 493

Anemia of Chronic Inflammation

Question 494

▪ The anemia is often corrected when the primary disease is resolved

Answer 494

Anemia of Chronic Inflammation

Question 495

▪ dec Transferrin (due to being a negative APR)

Answer 495

Anemia of Chronic Inflammation

Question 496

▪ Tuberculosis

Answer 496

Anemia of Chronic Inflammation

Question 497

▪ Lung abscess

Answer 497

Anemia of Chronic Inflammation

Question 498

▪ Bacterial endocarditis

Answer 498

Anemia of Chronic Inflammation

Question 499

▪ Neoplasms

Answer 499

Anemia of Chronic Inflammation

Question 500

▪ RA

Answer 500

Anemia of Chronic Inflammation

Question 501

▪ Rheumatic fever

Answer 501

Anemia of Chronic Inflammation

Question 502

▪ SLE

Answer 502

Anemia of Chronic Inflammation

Question 503

▪ Chronic liver disease

Answer 503

Anemia of Chronic Inflammation

Question 504

▪ Hormone produced by the liver to regulate body iron levels particularly absorption of iron in the intestine and release of iron from macrophages

Answer 504

Hepcidin

Question 505

▪ Iron-binding protein in the granules of the neutrophils

Answer 505

Lactoferrin

Question 506

▪ Prevents phagocytized bacteria from using intracellular iron

Answer 506

Lactoferrin

Question 507

▪ During infection and inflammation, inflammation is released into the plasma

Answer 507

Lactoferrin

Question 508

▪ Binds iron

Answer 508

Ferritin

Question 509

▪ Because developing RBCs do not have a ferritin receptor, this iron is unavailable for incorporation into hemoglobin

Answer 509

Ferritin

Question 510

▪ Because developing RBCs do not have a ferritin receptor, this iron is unavailable for incorporation into hemoglobin

Answer 510

Ferritin

Question 511

▪ A form of acquired porphyria and acquired Sideroblastic Anemia

Answer 511

Lead Poisoning

Question 512

▪ Children may be exposed to lead secondary to ingestion of leadcontaining paint

Answer 512

Lead Poisoning

Question 513

▪ Also associated with the use of improperly glazed pottery for cooking or eating

Answer 513

Lead Poisoning

Question 514

▪ Similar to Sideroblastic anemia (lead inhibits several enzymes needed in heme biosynthesis)

Answer 514

Lead Poisoning

Question 515

▪ Anemia, when present in lead poisoning, is most often normocytic and normochromic; however, with a chronic exposure to lead, a microcytic hypochromic clinical picture may be seen

Answer 515

Lead Poisoning

Question 516

▪ (+) Coarse basophilic stippling

Answer 516

Lead Poisoning

Question 517

Inherited disorder of defective heme synthesis

Answer 517

Porphyria

Question 518

Accumulation of porphyrin precursors

Answer 518

Porphyria

Question 519

Rare autosomal recessive disorder common in males

Answer 519

Hemochromatosis

Question 520

Abnormal iron deposition in the tissues causing “bronze skin pigmentation”

Answer 520

Hemochromatosis

Question 521

Associated with Bronze diabetes

Answer 521

Hemochromatosis

Question 522

Normal H/H
Inc Iron and transferrin saturation
Dec Transferrin

Answer 522

Hemochromatosis

Question 523

Caused by qualitative structural abnormalities of the globin chains that result from alteration of genetic sequence

Answer 523

Hemoglobinopathies

Question 524

Quantitative defect/reduction in globin chain synthesis

Answer 524

Thalassemia

Question 525

High incidence in Mediterranean descent

Answer 525

Thalassemia

Question 526

= sickle cell anemia

Answer 526

▪ Homozygous SS

Question 527

= sickle cell trait

Answer 527

▪ Heterozygous SS

Question 528

▪ Autosomal codominant

Answer 528

Sickle Cell Anemia

Question 529

▪ When fully oxygenated, hemoglobin S is fully soluble (reversible)

Answer 529

Sickle Cell Anemia

Question 530

▪ Sickling occurs when oxygen decreases at the tissue level

Answer 530

Sickle Cell Anemia

Question 531

▪ Provides resistance against P. falciparum

Answer 531

Sickle Cell Anemia

Question 532

▪ When oxygen is released from the molecule, a conformational change occurs, which results in polymerization of Hb molecule leading to the formation of tactoids or crystals which causes the cells to become rigid

Answer 532

Sickle Cell Anemia

Question 533

 Any situation that produces excessive deoxygenation of the RBC may cause painful sickle cell crises (e.g., infection, dehydration, strenuous exercise, obstetric delivery and high altitudes)

Answer 533

Sickle cell crises

Question 534

▪ Occur when rigid sickle cells increase the blood viscosity

Answer 534

Vaso occlusive crises

Question 535

▪ Occur when rigid sickle cells increase the blood viscosity

Answer 535

Vaso occlusive crises

Question 536

▪ Associated with the development of microthrombi, vascular occlusions, and microinfarction in the joints and extremities as well as in the major organs, which can cause organ failure

Answer 536

Vaso occlusive crises

Question 537

▪ Associated with the development of microthrombi, vascular occlusions, and microinfarction in the joints and extremities as well as in the major organs, which can cause organ failure

Answer 537

Vaso occlusive crises

Question 538

▪ Primary cause of death of patients with sickle cell anemia

Answer 538

Infectious crises

Question 539

▪ Causative agent: S. pneumoniae (common in children) ▪

Answer 539

Infectious crises

Question 540

▪ Also known as dactylitis

Answer 540

Hand-Foot syndrome

Question 541

▪ First sites affected by decreased blood flow are the small bones of hands and feet

Answer 541

Hand-Foot syndrome

Question 542

▪ Normocytic, normochromic anemia

Answer 542

Sickle Cell Anemia

Question 543

▪ (+) Sickle cell, Target cell, Ovalocyte, Schistocyte, Polychromasia, nRBC

Answer 543

Sickle Cell Anemia

Question 544

Sickle Cell Anemia
▪ [?] RDW
▪ [?] OFT, ESR
▪ [?] M:E ratio

Answer 544

Inc
Dec
Inc

Question 545

occurs when sickle cells become trapped in the splenic microcirculation.

Answer 545

Splenic sequestration

Question 546

▪ The spleen enlarges as more cells are trapped leading to [?] which may cause shock and death

Answer 546

hypovolemia

Question 547

▪ 2nd most common Hb variant

Answer 547

Hemoglobin C

Question 548

▪ tends to crystallize when dehydrated

Answer 548

Hemoglobin CC

Question 549

▪ Note: the cells most vulnerable to intracellular crystallization are[?] because they tend to lose water as they age

Answer 549

older RBCs

Question 550

▪ in MCHC
▪ Normocytic, normochromic anemia
▪ (+) Target cell, Spherocyte
▪ (+) Hb CC or SC crystal

Answer 550

Hemoglobin C disease

Question 551

▪ in MCHC
▪ Normocytic, normochromic anemia
▪ (+) Target cell, Spherocyte
▪ (+) Hb CC or SC crystal

Answer 551

Hemoglobin C disease

Question 552

▪ Caused by deletion of all four alpha globin genes (–/–) resulting in the production of hemoglobin Barts (γ4)

Answer 552

Bart’s Hydrops Fetalis

Question 553

▪ has high affinity for oxygen

Answer 553

Bart’s Hydrops Fetalis

Question 554

▪ The disorder is lethal; infants are usually stillborn or die within hours of birth

Answer 554

Bart’s Hydrops Fetalis

Question 555

▪ Hb Barts has high affinity for oxygen

Answer 555

Bart’s Hydrops Fetalis

Question 556

▪ Hb Barts has high affinity for oxygen

Answer 556

Bart’s Hydrops Fetalis

Question 557

▪ The disorder is lethal; infants are usually stillborn or die within hours of birth

Answer 557

Bart’s Hydrops Fetalis

Question 558

▪ Caused by deletion of 3 out of 4 alpha globin genes (–/-a)

Answer 558

Hb H Disease

Question 559

▪ This disorder results from the decreased synthesis of alpha chains and the resultant formation of the unstable hemoglobin, Hb H (β4)

Answer 559

Hb H Disease

Question 560

Hb H Disease ▪ At birth =

Answer 560

Inc Hb Barts (due to the presence of gamma globin chains)

Question 561

▪ Also known as alpha thalassemia trait

Answer 561

Alpha Thalassemia minor

Question 562

Hb H Disease ▪ Adult =

Answer 562

Inc Hb H (due to availability of beta globin and replaces Barts Hb

Question 563

▪ Caused by deletion of 2 out of 4 alpha globin genes

Answer 563

Alpha Thalassemia minor

Question 564

▪ Caused by deletion of 2 out of 4 alpha globin genes

Answer 564

Alpha Thalassemia minor

Question 565

▪ Heterozygous a0 (–/aa)
▪ Homozygous a+ (a-/a-)

Answer 565

Alpha Thalassemia minor

Question 566

▪ Also known as heterozygous alpha thalassemia

Answer 566

Silent carrier

Question 567

▪ Caused by deletion of 1 out of 4 alpha globin genes (-a/aa)

Answer 567

Silent carrier

Question 568

▪ Benign, and often discovered only during family studies

Answer 568

Silent carrier

Question 569

Results to reduced production of beta globin chains → excess production of alpha chain (unstable)

Answer 569

Beta Thalassemia

Question 570

▪ Also known as Cooley’s anemia/Mediterranean anemia

Answer 570

Thalassemia major

Question 571

▪ Beta globin chain synthesis is impaired

Answer 571

Thalassemia major

Question 572

▪ inc Hb A2

Answer 572

Thalassemia major

Question 573

Marked skeletal deformities with frontal bossing, cheek bone and jaw protrusion

Answer 573

Beta Thalassemia

Question 574

Characterized by increased levels of Hb F in adults in the absence of the usual hematologic features of thalassemia

Answer 574

Hereditary Persistence of Hemoglobin F

Question 575

1st most common Enzymopathies

Answer 575

G6PD Deficiency

Question 576

Most common RBC enzymopathy

Answer 576

G6PD Deficiency

Question 577

Produced in: HMP

Answer 577

G6PD Deficiency

Question 578

Triggers of hemolysis: antimalarial drugs, fava beans

Answer 578

G6PD Deficiency

Question 579

Classical finding: Heinz bodies

Answer 579

G6PD Deficiency

Question 580

2nd most common Enzymopathies

Answer 580

PK Deficiency

Question 581

Produced: EMP

Answer 581

PK Deficiency

Question 582

can lead to hemolytic anemia

Answer 582

PK deficiency

Question 583

Deficiency 3rd most common Enzymopathies

Answer 583

Pyrimidine-5-nucleotidase

Question 584

Substitution of glutamic acid to lysine at 6th position of the β-chain

Answer 584

Hemoglobin C

Question 585

Substitution of glutamic acid to lysine at 26th position of the β -chain

Answer 585

Hemoglobin E

Question 586

Substitution of asparagine to threonine at 102nd position of the β-chain

Answer 586

Hemoglobin Kansas

Question 587

Substitution of glutamic acid to lysine at 121st position of the β-chain

Answer 587

Hemoglobin O-Arab

Question 588

Also known as D-Punjab Substitution of glutamic acid to glycine at 121st position of the β-chain

Answer 588

Hemoglobin D-Los Angeles

Question 589

Also known as C-Georgetown

Answer 589

Hemoglobin C-Harlem

Question 590

Caused by two amino acid substitutions

Answer 590

Hemoglobin C-Harlem

Question 591

Substitution of glutamic acid to valine at 6th position and aspartic acid to asparagine at 73rd position of the β-chain

Answer 591

Hemoglobin C-Harlem

Question 592

Substitution of asparagine to lysine at 68th position of the alpha chain

Answer 592

Hemoglobin G-Philadelphia

Question 593

Substitution of arginine to leucine at 92nd position of the alpha chain

Answer 593

Hemoglobin Chesapeake

Question 594

Addition of 31 amino acids in the alpha chain

Answer 594

Hemoglobin Constant Spring

Question 595

Amino acid deletion

Answer 595

Hemoglobin Gun Hill

Question 596

Unstable hemoglobin

Answer 596

Hemoglobin Koln

Question 597

Protein 4.1 and Spectrin deficiency

Answer 597

Hereditary Spherocytosis

Question 598

(+) Autohemolysis test

Answer 598

Hereditary Spherocytosis

Question 599

Inc OFT

Answer 599

Hereditary Spherocytosis

Question 600

Protein 4.1 deficiency

Answer 600

Hereditary Elliptocytosis

Question 601

Spectrin deficiency

Answer 601

Hereditary Pyropoikilocytosis

Question 602

Commonly seen in burn patients

Answer 602

Hereditary Pyropoikilocytosis

Question 603

Band 3 deficiency

Answer 603

Southeast Asian Ovalocytosis

Question 604

Hemolytic anemia with dehydrated red blood cells

Answer 604

Hereditary Xerocytosis

Question 605

(+) stomatocytes, target cells, macrocytes

Answer 605

Hereditary Xerocytosis

Question 606

Also known as hydrocytosis

Answer 606

Hereditary Stomatocytosis

Question 607

Characterized by neurologic impairment and acanthocytes on the PBS

Answer 607

Neuroacanthocytosis

Question 608

Also known as hereditary acanthocytosis

Answer 608

Abetalipoproteinemia (Bassen-Kornzweig Syndrome)

Question 609

Due to absence of β-lipoprotein

Answer 609

Abetalipoproteinemia (Bassen-Kornzweig Syndrome)

Question 610

Characterized by malabsorption of fat, retinitis pigmentosa, neurologic damage and acanthocytosis

Answer 610

Abetalipoproteinemia (Bassen-Kornzweig Syndrome)

Question 611

Characterized by chorea, hyperkinesia, cognitive impairment, and neuropsychiatric symptoms

Answer 611

Chorea Acanthocytosis

Question 612

Due to KX gene mutation

Answer 612

McLeod syndrome

Question 613

– precursor for the production of Kell antigen

Answer 613

Kx substance

Question 614

▪ Also known as Marchiafava-Micheli syndrome

Answer 614

Paroxysmal Nocturnal Hemoglobinuria

Question 615

▪ Hemoglobinuria occurs at night when blood pH falls (acidic)

Answer 615

Paroxysmal Nocturnal Hemoglobinuria

Question 616

▪ Complication of PNH may progress to aplastic anemia

Answer 616

Paroxysmal Nocturnal Hemoglobinuria

Question 617

▪ Cause: stem cell mutation that results in circulating blood cells that lack CD55 and CD59

Answer 617

Paroxysmal Nocturnal Hemoglobinuria

Question 618

Paroxysmal Nocturnal Hemoglobinuria
▪ = DAF
▪ = MIRL

Answer 618

CD55

CD59

Question 619

are complement-inhibiting regulator proteins

Answer 619

▪ CD55 and CD59

Question 620

Paroxysmal Nocturnal Hemoglobinuria ▪ Screening test:

Answer 620

Sugar water test or sucrose hemolysis test

Question 621

Paroxysmal Nocturnal Hemoglobinuria ▪ Confirmatory test:

Answer 621

Acidified Serum Test (Ham’s test)

Question 622

▪ Dec LAP

Answer 622

Paroxysmal Nocturnal Hemoglobinuria

Question 623

Due to severe liver disease that develop a hemolytic anemia with acanthocyte

Answer 623

Spur cell anemia

Question 624

 A disease of small blood vessels

Answer 624

Microangiopathic Hemolytic Anemia

Question 625

 Can be a complication of one of several conditions in which there is a disturbance of the microvascular environment (DIC, TTP, HUS)

Answer 625

Microangiopathic Hemolytic Anemia

Question 626

▪ When there is extensive damage to vessel endothelium or exposure to compounds to initiate clotting (thromboplastic substances that encourage coagulation), DIC may follow

Answer 626

DIC

Question 627

▪ As a direct result of fibrin deposition along and across the vessel lumen, RBCs can be fragmented or destroyed as they are pushed through the vessel by the action of blood pressure and rapidly flowing circulation

Answer 627

DIC

Question 628

▪ (+) Schistocyte

Answer 628

DIC

Question 629

▪ (+) D-dimer

Answer 629

DIC

Question 630

▪ Also known as Moschkovitz syndrome

Answer 630

TTP

Question 631

▪ Due to ADAMTS13 deficiency

Answer 631

TTP

Question 632

▪ Most common in children

Answer 632

HUS

Question 633

▪ Involves acute intravascular hemolysis and renal failure

Answer 633

HUS

Question 634

▪ Causative agent: E. coli O157:H7

Answer 634

HUS

Question 635

▪ EHEC produces Shiga-like toxin

Answer 635

HUS

Question 636

▪ (+) Shistocytes, Burr cells, polychromasia

Answer 636

HUS

Question 637

▪ Inc BUN, Creatinine

Answer 637

HUS

Question 638

Autoimmune Hemolytic Anemia

Answer 638

WAIHA
CAIHA
PCH

Question 639

Alloimmune Hemolytic Anemia

Answer 639

HDN
HTR
DIHA

Question 640

Caused by Autoanti-P (biphasic hemolysin)

Answer 640

Paroxysmal Cold Hemoglobinuria

Question 641

Paroxysmal Cold Hemoglobinuria Diagnostic test:

Answer 641

Donath-Landsteiner Test

Question 642

Infection

Answer 642

Leishmaniasis Malaria Babesiosis B. bacilliformis

Question 643

Also known as Chronic Myeloproliferative Disorders

Answer 643

MYELOPROLIFERATIVE DISORDERS

Question 644

Splenomegaly is a common finding (extramedullary hematopoiesis)

Answer 644

MYELOPROLIFERATIVE DISORDERS

Question 645

Examples:
Polycythemia vera
Essential Thrombocythemia
Primary Myelofibrosis
CML

Answer 645

MYELOPROLIFERATIVE DISORDERS

Question 646

▪ Also known as Primary Thrombocytosis, Idiopathic Thrombocytosis

Answer 646

Essential Thrombocythemia

Question 647

▪ Characterized by a thrombocytosis of 1000 x 109/L with spontaneous aggregation of functionally abnormal platelets

Answer 647

Essential Thrombocythemia

Question 648

▪ Must be differentiated from secondary or reactive thrombocytosis

Answer 648

Essential Thrombocythemia

Question 649

▪ Due to mutation of JAK2 gene

Answer 649

Essential Thrombocythemia

Question 650

▪ Markedly increased platelet count

Answer 650

Essential Thrombocythemia

Question 651

▪ Bone marrow examination: megakaryocytes stick together (Glued together appearance)

Answer 651

Essential Thrombocythemia

Question 652

▪ Characterized by fibrosis and granulocytic hyperplasia of the bone marrow, with granulocytic and megakaryocytic proliferation in the liver and spleen

Answer 652

Primary Myelofibrosis Description

Question 653

▪ Hepatomegaly and splenomegaly are common (extramedullary hematopoiesis)

Answer 653

Primary Myelofibrosis

Question 654

▪ Due to mutation of JAK2 gene

Answer 654

Primary Myelofibrosis

Question 655

▪ Normocytic, normochromic anemia

Answer 655

Primary Myelofibrosis

Question 656

▪ Inc Reticulocyte count
▪ Inc WBC

Answer 656

Primary Myelofibrosis

Question 657

▪ PBS: Teardrop cell, Polychromatophilia, NRBC

Answer 657

Primary Myelofibrosis

Question 658

▪ Bone marrow examination: “dry tap” → presence of fibrotic tissues

Answer 658

Primary Myelofibrosis

Question 659

▪ A stem cell disorder affecting the granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines

Answer 659

Chronic Myelogenous Leukemia

Question 660

▪ Common in adults (between ages of 30 and 50)

Answer 660

Chronic Myelogenous Leukemia

Question 661

▪ A WBC count of 50,000-300,000/uL is often diagnostic of CML

Answer 661

Chronic Myelogenous Leukemia

Question 662

▪ Cause: translocation between the long arms of chromosome 9 and 22

Answer 662

Chronic Myelogenous Leukemia

Question 663

▪ Philadelphia chromosome: indicates good prognosis

Answer 663

Chronic Myelogenous Leukemia

Question 664

▪ Must be differentiated from Leukemoid reaction using LAP test

Answer 664

Chronic Myelogenous Leukemia

Question 665

▪ Inc RBC, WBC, and Platelet count

Answer 665

Chronic Myelogenous Leukemia

Question 666

▪ BM Examination: hypercellular bone marrow

Answer 666

Chronic Myelogenous Leukemia

Question 667

▪ Inc M:E ratio ▪ Inc LAP

Answer 667

Chronic Myelogenous Leukemia

Question 668

Formerly known as Refractory anemia

Answer 668

Myelodysplastic Syndrome

Question 669

Caused by proliferation of abnormal stem cells

Answer 669

Myelodysplastic Syndrome

Question 670

Poikilocytosis, Basophilic stippling, Howell-Jolly bodies, and Siderocytes

Answer 670

Myelodysplastic Syndrome

Question 671

French-American-British Classification of MDS

Answer 671

1. RARS 2. RAEB-t 3. RAEB 4. CMML

Question 672

 An abnormal, uncontrolled proliferation and accumulation of one or more of the hematopoietic cells

Answer 672

Leukemia

Question 673

 A disease of the blood forming tissues and the bone marrow

Answer 673

Leukemia

Question 674

Classification of Leukemia

Answer 674

Based on duration of the untreated disease
Based on number of WBC present
Based on WBC type involved

Question 675

Based on duration of the untreated disease
▪ Acute leukemia:
▪ Subacute leukemia:
▪ Chronic leukemia:

Answer 675

several days to 6 months

2 to 6 months

1-2 years

Question 676

Based on number of WBC present
▪ Leukemic leukemia:
▪ Subleukemic leukemia:
▪ Aleukemic leukemia:

Answer 676

> 15,000/uL

<15,000/uL w/ immature WBCs

<15,000/uL

Question 677

Based on WBC type involved
▪ Acute leukemia:
▪ Chronic leukemia:

Answer 677

predominance of blasts

predominance of mature WBCs

Question 678

Small cell, homogenous

Answer 678

L1

Question 679

Most common acute leukemia in children

Answer 679

L1

Question 680

Best prognosis

Answer 680

L1

Question 681

Large cell, heterogenous

Answer 681

L2

Question 682

Burkitt type

Answer 682

L3

Question 683

Large lymphocytes w/ basophilic cytoplasm and numerous vacuoles

Answer 683

L3

Question 684

Poor prognosis

Answer 684

L3

Question 685

Acute myeloblastic leukemia, minimally differentiated

Answer 685

M0

Question 686

Acute myeloblastic leukemia without maturation

Answer 686

M1

Question 687

Acute myeloblastic leukemia with maturation

Answer 687

M2

Question 688

Acute promyelocytic leukemia

Answer 688

M3

Question 689

Characterized by the presence of bowtie/butterfly appearance of nucleus

Answer 689

M3

Question 690

DIC is common

Answer 690

M3

Question 691

(+) FSP
(+) Faggot cells
PT and APTT: prolonged
Dec Fibrinogen

Answer 691

M3

Question 692

Acute myelomonocytic leukemia

Answer 692

M4

Question 693

Also known as Naegeli monocytic leukemia

Answer 693

M4

Question 694

Acute monocytic leukemia

Answer 694

M5

Question 695

Also known as Schilling leukemia

Answer 695

M5

Question 696

: ▪ Poorly differentiated leukemia

Answer 696

M5a

Question 697

▪ Nucleus: lacy chromatin w/ nucleoli

Answer 697

M5a

Question 698

▪ Well differentiated leukemia

Answer 698

M5b

Question 699

▪ Nucleus: cerebriform shape with nucleoli

Answer 699

M5b

Question 700

▪ Presence of all stages of monocytes in the peripheral blood

Answer 700

M5b

Question 701

Erythroleukemia

Answer 701

M6

Question 702

Also known as Di Guglielmo’s syndrome

Answer 702

M6

Question 703

Predominance of myeloblasts and erythroblasts in the peripheral blood

Answer 703

M6

Question 704

Acute megakaryocytic leukemia

Answer 704

M7

Question 705

Predominance of megakaryocytes

Answer 705

M7

Question 706

(+) PAS, ACP, platelet peroxidase

Answer 706

M7

Question 707

M1, M2, M3, M4

Answer 707

MPO
SBB
Specific esterase (Naphthyl AS-D chloroacetate)

Question 708

M1, M2, M3, M4

Answer 708

MPO
SBB
Specific esterase (Naphthyl AS-D chloroacetate)

Question 709

M4, M5, M6

Answer 709

Nonspecific esterase (Naphthyl Acetate and Butyrate)

Question 710

M5, M6, M7

Answer 710

PAS

Question 711

ALL: +
AML: -

Answer 711

Terminal deoxynucleotidyl transferase
PAS
Oil Red O

Question 712

ALL: -
AML: +

Answer 712

MPO
SBB

Question 713

Most affected lymphocytes are B-cells

Answer 713

Chronic Lymphocytic Leukemia

Question 714

Characterized by fragile lymphocytes

Answer 714

Chronic Lymphocytic Leukemia

Question 715

Smudge cells (formed during film preparation, thumbprint appearance)

Answer 715

Chronic Lymphocytic Leukemia

Question 716

(+) PAS

Answer 716

Chronic Lymphocytic Leukemia

Question 717

▪ A stem cell disorder affecting the granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines

Answer 717

Chronic Myelocytic Leukemia

Question 718

▪ Common in adults (between ages of 30 and 50)

Answer 718

Chronic Myelocytic Leukemia

Question 719

▪ A WBC count of 50,000-300,000/uL is often diagnostic of CML

Answer 719

Chronic Myelocytic Leukemia

Question 720

▪ Cause: translocation between the long arms of chromosome 9 and 22

Answer 720

Chronic Myelocytic Leukemia

Question 721

▪ Philadelphia chromosome: indicates good prognosis

Answer 721

Chronic Myelocytic Leukemia

Question 722

▪ Must be differentiated from Leukemoid reaction using LAP test

Answer 722

Chronic Myelocytic Leukemia

Question 723

▪ Inc RBC, WBC, and Platelet count
▪ BM Examination: hypercellular bone marrow
▪ Inc M:E ratio
▪ Dec LAP

Answer 723

▪ Chronic Myelocytic Leukemia

Question 724

▪ (+) Basket cells

Answer 724

Chronic Myelocytic Leukemia

Question 725

= nuclear remnants of granulocytic cells w/ netlike chromatin pattern

Answer 725

Basket cells

Question 726

= nuclear remnants of granulocytic cells w/ netlike chromatin pattern

Answer 726

Basket cells

Question 727

= nuclear remnants of granulocytic cells w/ netlike chromatin pattern

Answer 727

Basket cells

Question 728

Also known as Leukemic Reticuloendotheliosis

Answer 728

Hairy Cell Leukemia

Question 729

: lymphocyte w/ hairlike projections around the outer border

Answer 729

Hairy cell

Question 730

(+) TRAP

Answer 730

HCL

Question 731

 A group of malignant tumors of the lymphoid tissue

Answer 731

Lymphoma

Question 732

 Usually, blood and bone marrow are not involved

Answer 732

Lymphoma

Question 733

 Lymphoma Classification:

Answer 733

Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, Miscellaneous lymphoma

Question 734

Starts in one lymph node group and spreads in a predictable fashion to adjacent lymph nodes (unifocal)

Answer 734

Hodgkin’s Lymphoma

Question 735

Hodgkin’s Lymphoma Definitive diagnostic test:

Answer 735

Lymph node biopsy

Question 736

= classifies the different types of Hodgkin’s lymphoma

Answer 736

▪ Rye Classification

Question 737

= used to formulate the treatment plan

Answer 737

▪ Ann Arbor staging system

Question 738

Types of Hodgkin’s Lymphoma:

Answer 738

a. Nodular lymphocyte-predominant Hodgkin lymphoma
b. Classical Hodgkin lymphoma

Question 739

-predominant Hodgkin lymphoma

Answer 739

a. Nodular lymphocyte

Question 740

− presence of popcorn cell

Answer 740

a. Nodular lymphocyte

Question 741

Classical Hodgkin lymphoma − types:

Answer 741

✓ Lymphocytic predominant (best prognosis)
✓ Lymphocyte depleted (worst prognosis)
✓ Mixed cellularity
✓ Nodular sclerosis (most common type)

Question 742

− presence of Reed-Sternberg cell

Answer 742

b. Classical Hodgkin lymphoma

Question 743

Spreads in a much less predictable way

Answer 743

Non-Hodgkin’s Lymphoma

Question 744

Classified under Rappaport system (replaced by NCI)

Answer 744

Non-Hodgkin’s Lymphoma

Question 745

Non-Hodgkin’s Lymphoma Types:

Answer 745

✓ Well differentiated lymphocytic lymphoma
✓ Poorly-differentiated lymphocytic lymphoma
✓ Histiocytic lymphoma
✓ Mixed histiocytic-lymphocytic lymphoma

Question 746

▪ Caused by neoplastic T-cells that migrate into the skin

Answer 746

Mycosis fungoides

Question 747

▪ Affects primarily the T-cells

Answer 747

Mycosis fungoides

Question 748

▪ Classic symptoms: pruritus

Answer 748

Mycosis fungoides

Question 749

▪ Pautrier’s microabscesses = cluster of lymphocytes in the epidermis

Answer 749

Mycosis fungoides

Question 750

▪ NOTE: fungal infection is not present

Answer 750

Mycosis fungoides

Question 751

▪ Leukemic phase of mycosis fungoides

Answer 751

Sezary syndrome

Question 752

▪ (+) Sezary cells

Answer 752

Sezary syndrome

Question 753

▪ Prognosis is worst at this stage

Answer 753

Sezary syndrome

Question 754

▪ Also known as Kahler’s disease

Answer 754

Multiple Myeloma

Question 755

▪ Caused by excessive production of IgG

Answer 755

Multiple Myeloma

Question 756

▪ The homogenous protein synthesized by the abnormal clone may be complete immunoglobulins or light chains (kappa and lambda)

Answer 756

Multiple Myeloma

Question 757

▪ The homogenous protein synthesized by the abnormal clone may be complete immunoglobulins or light chains (kappa and lambda)

Answer 757

Multiple Myeloma

Question 758

▪ Idiopathic

Answer 758

Multiple Myeloma

Question 759

▪ Clonal proliferation begins in the bone marrow and multiple tumors appear as patchy infiltrates in skeletal structures producing osteoporosis and lytic bone disease

Answer 759

Multiple Myeloma

Question 760

▪ As the neoplastic mass grows, pathologic bone fractures and vertebral collapse may occur

Answer 760

Multiple Myeloma

Question 761

▪ (+) Bence Jones proteins in urine

Answer 761

Multiple Myeloma

Question 762

▪ PBS: Rouleaux formation, Dutcher bodies, Russell bodies

Answer 762

Multiple Myeloma

Question 763

▪ Caused by excessive production of IgM

Answer 763

Waldenstrom’s Macroglobulinemia

Question 764

▪ Monoclonal IgM may exhibit cryoglobulin activity demonstrated by precipitation or gel formation during refrigeration at 4’C and dissolve when heated

Answer 764

Waldenstrom’s Macroglobulinemia

Question 765

▪ May result to renal damage caused by deposition of IgM complexes

Answer 765

Waldenstrom’s Macroglobulinemia

Question 766

▪ Characterized by a WBC count of greater than 50,000/uL

Answer 766

Leukemoid Reaction

Question 767

▪ Resembles CML

Answer 767

Leukemoid Reaction

Question 768

Leukemoid Reaction ▪ Differential test:

Answer 768

LAP score

Question 769

▪ Inc LAP

Answer 769

Leukemoid Reaction

Question 770

▪ Also known as Leukoerythroblastic anemia

Answer 770

Leukoerythroblastic Reaction

Question 771

▪ (+) NRBCs and immature neutrophils in peripheral blood smear

Answer 771

Leukoerythroblastic Reaction

Question 772

▪ Caused by space-occupying disturbances of the bone marrow

Answer 772

Leukoerythroblastic Reaction

Question 773

▪ Caused by space-occupying disturbances of the bone marrow

Answer 773

Leukoerythroblastic Reaction

Question 774

Causes:
▪ Myelofibrosis w/ myeloid metaplasia
▪ Metastatic carcinoma
▪ Leukemia
▪ Multiple myeloma
▪ Gaucher disease

Answer 774

Leukoerythroblastic Reaction

Question 775

▪ Hyposegmented neutrophils (bilobed)

Answer 775

Pelger-Huet anomaly

Question 776

▪ The bilobed nuclei are commonly described as “pince-nez spectacles” or peanut or dumbbell shape

Answer 776

Pelger-Huet anomaly

Question 777

Pelger-Huet anomaly
▪ Homozygous =
▪ Heterozygous =

Answer 777

round nucleus

pince-nez nucleus (more common)

Question 778

▪ Also known as hereditary hypersegmentation of neutrophils

Answer 778

Undritz anomaly

Question 779

▪ Characterized by leukopenia, thrombocytopenia, giant platelets, and presence of gray-blue spindle-shaped inclusions in the cytoplasm of granulocytes and monocytes

Answer 779

May-Hegglin anomaly

Question 780

▪ NOTE: the cytoplasmic inclusion resembles Dohle bodies

Answer 780

May-Hegglin anomaly

Question 781

▪ Characterized by the presence of abnormally large azurophilic granules resembling severe toxic granulation in the cytoplasm of granulocyte, lymphocyte and monocyte

Answer 781

Alder-Reilly anomaly

Question 782

▪ Associated with mucopolysaccharidoses

Answer 782

Alder-Reilly anomaly

Question 783

▪ Random movement of phagocytes is normal, but chemotaxis (directional motility) is impaired

Answer 783

Job’s syndrome

Question 784

▪ As a result, bacteria have more time to multiply in the tissues

Answer 784

Job’s syndrome

Question 785

▪ Both random and directed movement of the phagocytes are impaired

Answer 785

Lazy Leukocyte syndrome

Question 786

▪ Presence of giant cytoplasmic granules in the phagocytes and lymphocytes

Answer 786

Chediak Higashi anomaly

Question 787

▪ Inability of phagocytes to produce superoxide and ROS

Answer 787

Chronic Granulomatous Disease

Question 788

▪ Due to NADPH oxidase

Answer 788

Chronic Granulomatous Disease

Question 789

▪ Diagnostic test: (-) NBT dye test

Answer 789

Chronic Granulomatous Disease

Question 790

▪ Also known as Alius-Grignaschi Anomaly

Answer 790

Myeloperoxidase deficiency

Question 791

Most common form of neutrophil abnormality

Answer 791

Myeloperoxidase deficiency

Question 792

▪ Function abnormality is not severe

Answer 792

Myeloperoxidase deficiency

Question 793

Neutrophil that contains a large spherical body in its cytoplasm ; SLE

Answer 793

LE cell

Question 794

Formed during LE cell preparation (May be confused with LE cell) ; Unknown

Answer 794

Tart cell

Question 795

Crumpled tissue paper appearance

Question 796

Macrophage with swollen cytoplasm composed of numerous small, uniform lipid droplets ; Niemann-Pick disease

Answer 796

Foam cell

Question 797

Abnormal plasma cell with immunoglobulin trapped in endoplasmic reticulum ; Plasma cell myeloma

Answer 797

Morula cell

Question 798

Nuclear remnants of granulocytic cells with netlike chromatin pattern Formed during blood film preparation ; CML

Answer 798

Basket cell

Question 799

Nuclear remnants of lymphocytes Formed during blood film preparation Thumbprint appearance ; CLL

Answer 799

Smudge cell

Question 800

Large lymphoid cells with a bilobed nucleus with prominent eosinophilic nucleoli and abundant cytoplasm (owl’s eye appearance) ; Hodgkin’s lymphoma

Answer 800

Reed-Sternberg cell

Question 801

Large lymphoid cells with abundant cytoplasm and vesicular multilobed nuclei ; Nodular Lymphocyte ; Predominant-Hodgkin’s lymphoma

Answer 801

Popcorn cell

Question 802

Plasma cell with abundant cytoplasm with a reddish tinge of ribosomal protein ; IgA myeloma

Answer 802

Flame cell

Question 803

Lymphocyte with hairlike projections around the outer border ; HCL

Answer 803

Hairy cell

Question 804

Contains bundles of Auer rods in the cytoplasm ; AML M3/APL

Answer 804

Faggot cell

Question 805

Also known as Reactive lymphocytes, atypical lymphocytes, stress lymphocytes, virocytes, variant lymphocytes, transformed lymphocytes ; Non-malignant reactive disorders

Answer 805

Downey cells

Question 806

✓ Type 1 –

Answer 806

Turk’s cell (also known as plasmacytoid lymphocyte, Turk’s irritation cell)

Question 807

✓ Type 2 –

Answer 807

Infectious mononucleosis cell (Flared skirt appearance)

Question 808

✓ Type 3 –

Answer 808

Transformed/Reticular lymphocytes

Question 809

▪ Pale blue, round or elongated bodies

Answer 809

Dohle bodies

Question 810

▪ Consists of rRNA

Answer 810

Dohle bodies

Question 811

▪ Found in neutrophils

Answer 811

Dohle bodies

Question 812

▪ Associated with pregnancy, infection, poisoning, burn and surgery

Answer 812

Dohle bodies

Question 813

▪ Resembles May-Hegglin bodies

Answer 813

Dohle bodies

Question 814

▪ Larger than Dohle bodies

Answer 814

May-Hegglin bodies

Question 815

▪ Consists of mRNA

Answer 815

May-Hegglin bodies

Question 816

▪ Found in granulocytes and monocytes

Answer 816

May-Hegglin bodies

Question 817

▪ Heavy, coarse, blue-black granulation of the leukocytes

Answer 817

Alder-Reilly bodies

Question 818

▪ Resembles toxic granules

Answer 818

Alder-Reilly bodies

Question 819

▪ Associated with Hurler’s and Hunter’s syndrome and Alder-Reilly Anomaly

Answer 819

Alder-Reilly bodies

Question 820

▪ Dark blue-black cytoplasmic granules in the neutrophil

Answer 820

Toxic granules

Question 821

▪ Composed of primary granules

Answer 821

Toxic granules

Question 822

▪ Associated with severe infection

Answer 822

Toxic granules

Question 823

▪ Results when the degenerating cytoplasm begins to acquire holes or as the result of active phagocytosis

Answer 823

Vacuolation

Question 824

▪ Associated with infection

Answer 824

Vacuolation

Question 825

▪ Rod-like bodies representing aggregated primary granules that stain a reddish purple

Answer 825

Auer rods

Question 826

▪ Associated with AML

Answer 826

Auer rods

Question 827

▪ Stimulates vasodilation

Answer 827

Prostacyclin (PGI2)

Adenosine

Question 828

Reduces blood flow rate

Answer 828

Prostacyclin (PGI2)

Adenosine

Question 829

Inhibits platelet activation

Answer 829

Prostacyclin (PGI2)

Question 830

Anticoagulant

Answer 830

Prostacyclin (PGI2)

Thrombomodulin

Heparan sulfate

Question 831

Fibrinolytic

Answer 831

Thrombomodulin

tPA

Question 832

Coagulation

Answer 832

von Willebrand factor

Question 833

▪ Endothelial receptor for thrombin

Answer 833

Thrombomodulin

Question 834

▪ Binds and inactivates thrombin and enhances anticoagulant and fibrinolytic action of Protein C

Answer 834

Thrombomodulin

Question 835

▪ Coats the endothelial cell surface and weakly enhances the activity of antithrombin-III

Answer 835

Heparan sulfate

Question 836

▪ Converts plasminogen to plasmin which plays an important role in fibrinolysis

Answer 836

tPA

Question 837

▪ NOTE: released only on appropriate stimulus, such as vessel injury, to prevent excessive clot formation at the site of tissue injury

Answer 837

tPA

Question 838

tPA ▪ NOTE: released only on appropriate stimulus, such as vessel injury, to prevent excessive clot formation at the site of tissue injury

Question 839

▪ Secreted by endothelium

Answer 839

von Willebrand factor

Question 840

▪ Required for platelet adhesion

Answer 840

von Willebrand factor

Question 841

✓ Synthesized by endothelial cells, and megakaryocytes

Answer 841

von Willebrand Factor

Question 842

✓ Stored in endothelial cells (Weibel-Palade bodies) and platelets

Answer 842

von Willebrand Factor

Question 843

: entire molecule as it circulates in the plasma

Answer 843

✓ VIII/vWF

Question 844

– portion of molecule responsible for binding to endothelium and supporting normal platelet adhesion and function

Answer 844

✓ VIII:vWF

Question 845

– portion of molecule participating in intrinsic pathway

Answer 845

✓ VIII:C

Question 846

✓ Labile factors:

Answer 846

V and VIII

Question 847

✓ Activated by cold temperature:

Answer 847

VII and XI

Question 848

✓ Fibrinogen is the most abundant clotting factor with a normal value of

Answer 848

200-400 mg/dL

Question 849

✓ Serine protease:

Answer 849

II, X, VII, IX, XII, XI, and PK

Question 850

✓ Prothrombinase:

Answer 850

Xa-Va

Question 851

Intrinsic Tenase:

Answer 851

VIII-IX

Question 852

Extrinsic Tenase:

Answer 852

VII-III-IV

Question 853

: central regulatory component of coagulation (can inhibit or accelerate coagulation)

Answer 853

Thrombin (IIa)

Question 854

▪ Extrinsic pathway:

Answer 854

III and VII

Question 855

▪ Intrinsic pathway:

Answer 855

XII, XI, IX, VIII

Question 856

▪ Common pathway:

Answer 856

I, II, V, X

Question 857

▪ Fibrinogen group:

Answer 857

I, V, VIII, XIII

Question 858

▪ Prothrombin group (Vitamin K dependent):

Answer 858

II, VII, IX, X

Question 859

▪ Contact group:

Answer 859

XI, XII, PK, HMWK

Question 860

▪ A transmembrane receptor for Factor VIIa

Answer 860

Tissue factor (thromboplastin)

Question 861

= mixture of tissue factor and phospholipid

Answer 861

▪ Tissue thromboplastin

Question 862

▪ Found on extravascular cells such as fibroblasts and smooth muscle cells (not found in endothelial cells under normal conditions)

Answer 862

Tissue factor (thromboplastin)

Question 863

▪ High levels are found in brain, lung, heart, kidneys, and testes

Answer 863

Tissue factor (thromboplastin)

Question 864

▪ Produced by B. fragilis and E. coli

Answer 864

Vitamin K

Question 865

▪ Found in green leafy vegetables

Answer 865

Vitamin K

Question 866

▪ Catalyzes an essential post-translational modification of the prothrombin group proteins

Answer 866

Vitamin K

Question 867

▪ Vitamin K dependent factors:

Answer 867

o II, VII, IX, X
o Protein C, S, Z

Question 868

▪ Participates in platelet adhesion and transports Factor VIII

Answer 868

von Willebrand Factor

Question 869

▪ Large multimeric glycoprotein

Answer 869

von Willebrand Factor

Question 870

▪ Composed of multiple subunits of 240,000 Da each

Answer 870

von Willebrand Factor

Question 871

▪ The subunits are produced by endothelial cells and megakaryocytes, where they combine to form multimers that range from 600,000 to 20,000,000 Da

Answer 871

von Willebrand Factor

Question 872

▪ Once released into the plasma, they are normally degraded into small-multimers by vWF-cleaving protease called ADAMTS13 (a disintegrin-like metalloprotease with a thrombospondin type 1 motif, member 13)

Answer 872

von Willebrand Factor

Question 873

= associated with abnormally large vWF

Answer 873

▪ TTP

Question 874

= associated with abnormally large vWF

Answer 874

▪ TTP

Question 875

▪ Required for the assembly of coagulation complexes

Answer 875

Calcium

Question 876

▪ Expressed by vascular endothelial cells

Answer 876

Thrombomodulin

Question 877

▪ Cofactor of thrombin

Answer 877

Thrombomodulin

Question 878

= activates Protein C

Answer 878

▪ Thrombomodulin + Thrombin

Question 879

: a coagulation inhibitory protein, and thrombin activatable fibrinolysis inhibitor (TAFI)

Answer 879

▪ Protein C

Question 880

▪ Once thrombin is bound to thrombomodulin, it loses its procoagulant ability to activate factors V and VII, and through the activation of Protein C, leads to the destruction of factors V and VII, thus suppressing further generation of thrombin

Answer 880

Thrombomodulin

Question 881

▪ Considered as the key protease of coagulation pathway

Answer 881

Thrombin

Question 882

▪ Primary function is to cleave fibrinopeptidases A and B from the alpha and beta chains of fibrinogen molecule, triggering spontaneous polymerization

Answer 882

Thrombin

Question 883

✓ Activates cofactors V, VIII, and IX

Answer 883

Thrombin

Question 884

✓ Activates factor XIII

Answer 884

Thrombin

Question 885

✓ Initiates platelet aggregation

Answer 885

Thrombin

Question 886

✓ Activates TAFI to suppress fibrinolysis

Answer 886

Thrombin

Question 887

Principal regulator of tissue factor pathway

Answer 887

Tissue factor pathway inhibitor

Question 888

→ Protein C → Activated Protein C

Answer 888

Thrombin + Thrombomodulin

Question 889

→ inactivates factor Va and VIIIa

Answer 889

Protein S

Question 890

Antithrombin and other serine protease inhibitors (Serpins)

Answer 890

Antithrombin
Heparin cofactor II
Protein Z-dependent protease inhibitor (ZPI)
Protein C inhibitor
a1-antitrypsin
a2-macroglobulin
a-2-antiplasmin
PAI-1

Question 891

Inhibits thrombin

Answer 891

Antithrombin III

Question 892

Synthesized by the liver

Answer 892

Antithrombin III

Question 893

SERPIN

Answer 893

Antithrombin III

Heparin cofactor II

Question 894

Inactivates thrombin

Answer 894

Heparin cofactor II

Question 895

Potent inhibitor of factor Xa

Answer 895

Protein Z-dependent protease inhibitor (ZPI)

Question 896

Final stage of coagulation

Answer 896

Fibrinolysis

Question 897

Dependent on plasmin

Answer 897

Fibrinolysis

Question 898

Destroys fibrinogen, fibrin, factor V, and factor VIII

Answer 898

Plasmin

Question 899

Not normally present in the blood in an active form

Answer 899

Plasmin

Question 900

Zymogen of plasmin

Answer 900

Plasminogen

Question 901

Normally present in the plasma

Answer 901

Plasminogen

Question 902

Homologous to Lp (a)

Answer 902

Plasminogen

Question 903

Released in vivo by endothelial cell damage

Answer 903

Tissue plasminogen activator

Question 904

Activates plasminogen

Answer 904

Tissue plasminogen activator

Question 905

Activates plasmin

Answer 905

Urokinase plasminogen activator

Question 906

May be administered to a patient to activate plasminogen

Answer 906

Urokinase plasminogen activator

Question 907

Activates plasminogen

Answer 907

Streptokinase

Question 908

Early degradation products:

Answer 908

FDP/FSP

X, Y

Question 909

Late degradation products:

Answer 909

FDP/FSP

D (D-dimer) and E

Question 910

Indicates fibrin degradation products

Answer 910

D-dimer

Question 911

Marker of thrombosis and fibrinolysis

Answer 911

D-dimer

Question 912

Produced by digestion of either fibrin or fibrinogen by plasmin

Answer 912

Fragment X, Y, E

Question 913

o Purplish red, pinpoint hemorrhagic spots caused by loss of capillary ability to withstand normal blood pressure and trauma

Answer 913

▪ Petechiae

Question 914

o Size: <3 mm

Answer 914

▪ Petechiae

Question 915

o Produced by hemorrhage of blood into small areas of skin, mucous membrane, and other tissues

Answer 915

▪ Purpura

Question 916

▪ o Size: <1 cm

Answer 916

Purpura

Question 917

o Form of purpura in which blood escapes into large areas of the skin or mucous membrane but not into deep tissues

Answer 917

▪ Ecchymosis

Question 918

o Size: >3 cm

Answer 918

▪ Ecchymosis

Question 919

▪ Autosomal Dominant

Answer 919

Ehlers-Danlos Syndrome

Hereditary hemorrhagic telangiectasia

Gray Platelet Syndrome

Question 920

▪ Characterized by hyperextensible joints and hyperelastic skin

Answer 920

Ehlers-Danlos Syndrome

Question 921

▪ Autosomal Recessive

Answer 921

Pseudoxanthoma elasticum

Bernard-Soulier Syndrome

Glanzmann’s Thrombasthenia

Hermansky-Pudlak Syndrome

Chediak-Higashi Anomaly

Thrombocytopenia w/ absent Radii

Question 922

▪ The connective tissue elastic fibers in small arteries are calcified and structurally abnormal

Answer 922

Pseudoxanthoma elasticum

Question 923

▪ Also known as Rendu-Osler-Weber syndrome

Answer 923

Hereditary hemorrhagic telangiectasia

Question 924

▪ Characterized by vascular malformations and skin lesions called telangiectasias

Answer 924

Hereditary hemorrhagic telangiectasia

Question 925

▪ Also known as Kasabach-Merritt syndrome

Answer 925

Congenital hemangiomathrombocytopenia syndrome

Question 926

▪ Associated with tumors composed of vessels that commonly swell and bleed at the surface

Answer 926

Congenital hemangiomathrombocytopenia syndrome

Question 927

▪ Formation of fibrin clots, platelet consumption, and RBC destruction secondary to vascular obstruction occur at the site of tumor

Answer 927

Congenital hemangiomathrombocytopenia syndrome

Question 928

▪ Also known as Scurvy

Answer 928

Vitamin C deficiency

Question 929

▪ Vitamin C is required for the formation of intact structure of the vascular basement membrane

Answer 929

Vitamin C deficiency

Question 930

▪ Gingival bleeding and hemorrhage into subcutaneous tissues and muscles are common finding

Answer 930

Vitamin C deficiency

Question 931

▪ Acquired and chronic disorder of the elderly causing abnormalities in connective tissues

Answer 931

Senile Purpura

Question 932

▪ The aging process brings about a degeneration of collagen, elastin, and subcutaneous fat

Answer 932

Senile Purpura

Question 933

▪ Common in elderly men

Answer 933

Senile Purpura

Question 934

▪ Purpura associated with abdominal pain secondary to GIT bleeding

Answer 934

Henoch-Schonlein Purpura

Question 935

▪ Abdominal and joint pain related to allergic purpura

Answer 935

Henoch-Schonlein Purpura

Question 936

▪ Common in children

Answer 936

Henoch-Schonlein Purpura

Question 937

▪ Caused by lack of expression of Gp Ib/IX complex on the platelet surface

Answer 937

Bernard-Soulier Syndrome

Question 938

▪ Characterized by large platelets

Answer 938

Bernard-Soulier Syndrome

Question 939

Lab Findings:
▪ Giant platelets
▪ BT: Prolonged
▪ Platelet Aggregation studies: Ristocetin is abnormal

Answer 939

Bernard-Soulier Syndrome

Question 940

▪ Lacks vWF

Answer 940

von Willebrand Disease

Question 941

Lab Findings:
▪ APTT, BT, TT, CT = Prolonged
▪ Platelet Aggregation studies: Ristocetin is abnormal
▪ Treatment of choice: cryoprecipitate, DDAVP

Answer 941

von Willebrand Disease

Question 942

▪ Lacks Gp IIb-IIIa

Answer 942

Glanzmann’s Thrombasthenia

Question 943

▪ Clot Retraction: Abnormal

Answer 943

Glanzmann’s Thrombasthenia

Question 944

▪ BT: Prolonged

Answer 944

Glanzmann’s Thrombasthenia

Question 945

▪ Platelet aggregation studies: ADP, Collagen, and Epinephrine are abnormal

Answer 945

Glanzmann’s Thrombasthenia

Question 946

▪ Lacks alpha granules

Answer 946

Gray Platelet Syndrome

Question 947

▪ Giant platelets

Answer 947

Gray Platelet Syndrome

Question 948

▪ Platelets are gray or blue-gray

Answer 948

Gray Platelet Syndrome

Question 949

▪ Lacks dense granules

Answer 949

Hermansky-Pudlak Syndrome

Question 950

▪ Triad of oculocutaneous albinism, bleeding tendency associated with abnormal platelet function, and accumulation of ceroid-like pigment in macrophages

Answer 950

Hermansky-Pudlak Syndrome

Question 951

▪ Lacks dense granules

Answer 951

Chediak-Higashi Anomaly

Question 952

▪ Characterized by albinism, recurrent infection, and giant lysosomes

Answer 952

Chediak-Higashi Anomaly

Question 953

▪ Decreased delta granules

Answer 953

Wiskott-Aldrich Syndrome

Question 954

▪ Platelets are small

Answer 954

Wiskott-Aldrich Syndrome

Question 955

▪ Triad of thrombocytopenia, recurrent infection and eczema

Answer 955

Wiskott-Aldrich Syndrome

Question 956

▪ X-linked recessive

Answer 956

Wiskott-Aldrich Syndrome

Question 957

▪ Platelets have structural defects in delta granules

Answer 957

Thrombocytopenia w/ absent Radii

Question 958

▪ Characterized by congenital absence of the radial bones

Answer 958

Thrombocytopenia w/ absent Radii

Question 959

 inhibits the synthesis of cyclooxygenase

Answer 959

Aspirin

Question 960

 inhibits platelet aggregation

Answer 960

Aspirin

Question 961

 most common acquired platelet disorder

Answer 961

Aspirin

Question 962

▪ Autoimmune disorder

Answer 962

ITP

Question 963

▪ Develops after transfusion of platelet containing blood products

Answer 963

PTP

Question 964

▪ Also known as Moschowitz syndrome

Answer 964

TTP

Question 965

▪ Characterized by triad of microangiopathic hemolytic anemia, thrombocytopenia, and neurologic abnormalities

Answer 965

TTP

Question 966

▪ Due to ADAMTS13 deficiency

Answer 966

TTP

Question 967

− Inherited TTP − Severe

Answer 967

 Upshaw-Schulman Syndrome

Question 968

− Caused by mutation in the ADAMTS13 gene

Answer 968

 Upshaw-Schulman Syndrome

Question 969

− Caused by autoantibodies

Answer 969

 Idiopathic TTP

Question 970

− triggered by infections, pregnancy, surgery, trauma, inflammation, and disseminated malignancy

Answer 970

 Secondary TTP

Question 971

− Trimethoprim, Ticlopidine, Quinine drugs

Answer 971

 Secondary TTP

Question 972

▪ Resembles TTP

Answer 972

HUS

Question 973

▪ Common in children

Answer 973

HUS

Question 974

▪ Due to E. coli infection

Answer 974

HUS

Question 975

▪ Similar to TTP

Answer 975

DIC

Question 976

▪ Thrombi are primarily composed of platelets and fibrinogen

Answer 976

DIC

Question 977

▪ (+) D-dimer

Answer 977

DIC

Question 978

▪ Due to massive blood transfusion (effect is temporary)

Answer 978

Dilutional

Question 979

▪ Rationale: stored blood contains platelets whose viability is severely impaired by the effects of storage and temperature. Under these conditions, the damaged platelets are rapidly sequestered by the RES of the patient resulting to thrombocytopenia

Answer 979

Dilutional

Question 980

Due to uncontrolled proliferation of platelets (e.g., PV, ET)

Answer 980

Primary Thrombocytosis

Question 981

Splenectomy

Answer 981

Secondary Thrombocytosis (Reactive)

Question 982

▪ Do not demonstrate clinical bleeding

Answer 982

PK deficiency

Question 983

▪ May be vulnerable to thrombotic tendencies

Answer 983

PK deficiency

Question 984

▪ Prolonged APTT

Answer 984

PK deficiency

HMWK deficiency

Factor XII deficiency

Factor XI deficiency

Factor VIII deficiency

Question 985

▪ Prolonged APTT, PT, Stypven time

Answer 985

Factor X deficiency

Question 986

▪ Prolonged APTT and PT

Answer 986

Factor IX deficiency

Factor V deficiency

Factor II deficiency

Factor I deficiency

Question 987

▪ 5M Urea: Abnormal

Answer 987

Factor XIII deficiency

Question 988

▪ Do not manifest bleeding disorder

Answer 988

Factor XII deficiency

Question 989

▪ May be vulnerable to thrombosis

Answer 989

Factor XII deficiency

Question 990

Confirmatory test: Factor XII assay

Answer 990

Factor XII deficiency

Question 991

▪ Hemophilia C

Answer 991

Factor XI deficiency

Question 992

▪ Also known as Hemophilia B, Christmas Disease, Rosenthal syndrome

Answer 992

Factor IX deficiency

Question 993

▪ Also known as Hemophilia A or Classic hemophilia or Royal disease

Answer 993

Factor VIII deficiency

Question 994

▪ X-linked disorder (males are affected)

Answer 994

Factor VIII deficiency

Question 995

▪ Also known as Owren’s disease or Parahemophilia

Answer 995

Factor V deficiency

Question 996

▪ Screening test for primary hemostasis

Answer 996

Bleeding Time

Question 997

▪ Principle: the time it takes for a standard wound to stop bleeding

Answer 997

Bleeding Time

Question 998

▪ NV: 2 to 4 minutes

Answer 998

Bleeding Time

Question 999

– earlobe was punctured (not accurate, obsolete)

Answer 999

▪ Duke’s method

Question 1000

– uses blood pressure cuff (40 mmHg)

Answer 1000

▪ Ivy method

Question 1001

– standardization of wound

Answer 1001

▪ Mielke

Question 1002

▪ For primary hemostasis

Answer 1002

Whole Blood Clotting Time

Question 1003

▪ Principle: when venous blood is put into a foreign surface, it will form a solid clot

Answer 1003

Whole Blood Clotting Time

Question 1004

▪ Lee and White method

Answer 1004

Whole Blood Clotting Time

Question 1005

 NV: 5-15 minutes

Answer 1005

Whole Blood Clotting Time

Question 1006

 Uses 75 x 100 mm test tube

Answer 1006

Whole Blood Clotting Time

Question 1007

 Visual detection of fibrin clot formation

Answer 1007

▪ Tilt tube method

Question 1008

▪ Also known as Capillary Fragility Test ; Used to measure capillary fragility

Answer 1008

Tourniquet Test

Question 1009

(+) result: formation of petechiae

Answer 1009

Tourniquet Test ▪

Question 1010

Tourniquet Test ▪ Grading:
1+ =
2+ =
3+ =
4+ =

Answer 1010

few petechiae on the anterior part of the forearm

many petechiae on the anterior part of the forearm

multiple petechiae over the whole arm and back of the arm

confluent petechiae on the arm and back of the hand

Question 1011

▪ For platelet aggregation in vitro

Answer 1011

Platelet aggregometry

Question 1012

▪ Reagents used: ADP, Collagen, Epinephrine, Ristocetin

Answer 1012

Platelet aggregometry

Question 1013

▪ For extrinsic and common pathway

Answer 1013

Prothrombin Time

Question 1014

▪ Used to monitor oral anticoagulants (Warfarin, Coumadin, Coumarin)

Answer 1014

Prothrombin Time

Question 1015

prolonged if fibrinogen level is <80 mg/dL

Answer 1015

Prothrombin Time

Question 1016

▪ PT reagent:

Answer 1016

 Thromboplastin (rabbit brain or lung tissue)
 Calcium chloride (CaCl2)

Question 1017

 Calculation made to standardize PT ; It is based on ratio of patient’s PT and normal mean PT

ISI – assigned by the manufacturer

Answer 1017

Prothrombin Time ▪ INR

Question 1018

▪ NV: 10-14 seconds

Answer 1018

Prothrombin Time

Question 1019

▪ For intrinsic and common pathway

Answer 1019

Activated Partial Thromboplastin Time

Question 1020

▪ Used to monitor heparin therapy

Answer 1020

Activated Partial Thromboplastin Time

Question 1021

▪ APTT Reagent:

Answer 1021

 Platelet substitute (phospholipid)
 Activator (Kaolin, Celite, Silica, Ellagic acid)

Question 1022

▪ NV: 20-45 seconds

Answer 1022

Activated Partial Thromboplastin Time

Question 1023

▪ Also known as Russell’s viper venom time

Answer 1023

Stypven Time

Question 1024

▪ For common pathway

Answer 1024

Stypven Time

Question 1025

Stypven Time ▪ Reagent:

Answer 1025

Russell’s viper venom is obtained from the snake Vipera russelli

Question 1026

▪ For fibrinogen

Answer 1026

Thrombin Time

Question 1027

▪ NOTE: affected by heparin therapy

Answer 1027

Thrombin Time

Question 1028

▪ Sensitive test in detecting heparin inhibition

Answer 1028

Thrombin Time

Question 1029

▪ Prolonged Thrombin time is noted when fibrinogen level is below

Answer 1029

75 to 100 mg/dL

Question 1030

▪ For fibrinogen

Answer 1030

Reptilase Time

Question 1031

▪ NOTE: NOT affected by heparin therapy

Answer 1031

Reptilase Time

Question 1032

is an enzyme found in the venom of Bothrops atrox snake

Answer 1032

▪ Reptilase

Question 1033

▪ Also known as Plasma Clotting Time

Answer 1033

Plasma Recalcification Time

Question 1034

▪ For intrinsic pathway

Answer 1034

Plasma Recalcification Time

Question 1035

▪ For Factor XIII

Answer 1035

Duckert’s test

Question 1036

▪ Reagent: 5M Urea and 1% Monochloroacetic acid

Answer 1036

Duckert’s test

Question 1037

▪ (+) Factor XIII deficiency: the clot is dissolved in the presence of 5M Urea

Answer 1037

Duckert’s test

Question 1038

▪ NOTE: a clot that has not stabilized by Factor XIII is soluble to 5M Urea

Answer 1038

Duckert’s test

Question 1039

▪ Screening test for DIC

Answer 1039

Fibrinosticon

Question 1040

▪ The presence of crosslinked D-dimer indicates that a stable fibrin clot has been lysed

Answer 1040

Fibrinosticon

Question 1041

▪ Screening test for DIC

Answer 1041

Ethanol Gelation Test

Question 1042

▪ Used to detect fibrin monomers in the plasma

Answer 1042

Ethanol Gelation Test

Question 1043

▪ Used to differentiate DIC from primary fibrinolysis

Answer 1043

Ethanol Gelation Test

Question 1044

Principle: ▪ During the process of DIC, the level of fibrin monomer (product of fibrinogen conversion to fibrin) in the blood increases

Answer 1044

Ethanol Gelation Test

Question 1045

▪ NaOH is added in the plasma to increase the pH to 7.70 (if pH is below 7.70, this will cause precipitation of fibrinogen instead of fibrin monomer)

Answer 1045

Ethanol Gelation Test

Question 1046

▪ Ethyl alcohol will cause precipitation of any fibrin monomers

Answer 1046

Ethanol Gelation Test

Question 1047

▪ Used to detect the presence of fibrin monomers

Answer 1047

Protamine Sulfate

Question 1048

▪ NOTE: normally, there should be no fibrin monomers present in the plasma

Answer 1048

Protamine Sulfate

Question 1049

▪ Screening test for fibrinolytic activity

Answer 1049

Euglobulin clot lysis time

Question 1050

▪ Clot lysis in less than 1 hour indicates abnormal fibrinolytic activity

Answer 1050

Euglobulin clot lysis time

Question 1051

▪ Used for the detection of lupus anticoagulant

Answer 1051

Platelet neutralization test

Question 1052

▪ Also known as Mixing studies

Answer 1052

Substitution Test

Question 1053

Used to identify specific factor deficiency

Answer 1053

▪ Substitution Test

Question 1054

▪ A specific factor deficiency may be identified by mixing correction reagents with a patient’s plasma and then performing PT and APTT (1:1 dilution)

Answer 1054

Substitution Test