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Hematology > Cumulative From Exam (I-II-III) > Flashcards

Cumulative From Exam (I-II-III) Flashcards

1
Q

What is the disorder when an enzyme is missing in heme synthesis?

A
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2
Q

Multi fill in the blank - Aplastic anemia+ hemolytic anemia (lab findings)

A
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3
Q

Deficiency disorder hemostasis RBC+WBC

Propitious anemia B12 , Intrinsic factor

A
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4
Q

APTT - monitors heparin

A

Alternative anti factor Xa - Assay

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5
Q

PT - Monitors Warfarin/Coumadin

A

Withholds vitamin K form prothrombin proteins

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6
Q

average blood volume

A

4-6 L in adult

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7
Q

blood pH

A

7.35-7.45

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8
Q

blood specific gravity

A

1.049-1.065

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9
Q

blood composition

A

55% plasma/serum (fluid)

45% formed elements (cells)

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10
Q

Serum

A

NO anticoagulant used, some clotting factors consumed

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11
Q

In serum, there are proteins …

A

55% are albumin

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12
Q

WBC count

A

4.8-10.8x10^3

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13
Q

Major blood functions

A

Transport oxygen to cells
Transport carbon dioxide and wastes away
Provide defense
Regulate body pH , body temp, fluid balance

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14
Q

homeostasis

A

maintaining a constant environment equilibrium

(major blood function)

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15
Q

Platelets (thrombocyte)

A

Size: 1-4 um
No nucleus
Life span: 10 days
Function: Hemostasis –> blood clots, and arrested bleeding

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16
Q

Neutrophils

A

Inflammation and phagocytosis - 1st line of defense against infections.

2-5 lobes connected by thin filament, segmentation allows passing through openings between lining cells

life span: 6-10hrs

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17
Q

Eosinophil

A

Defense in parasitic and fungal infections.

Secondary granules (red/orange)

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18
Q

Basophil

A

Mediates allergic reactions

smallest WBC in circulation

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19
Q

histamine

A

vasoconstriction

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20
Q

heparin

A

blood thinner

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21
Q

lymphocyte functions

A

Immune response and viral infections

varies in size and shape

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22
Q

Monocytes function

A

Phagocytosis

Nucleus: horseshoe, lima bean, convoluted

Cytoplasm: Ground glass - cloudy

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23
Q

mature RBC - Erythrocyte

A

Major function: Oxygen transport from lungs to tissue

6-8um Biconcave disc –> max SA for gaseous exchange

No nucleus - no mitosis

Hgb is 95% dry weight

120 days life span.

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24
Q

Organs within Hematopoietic system

A

BM
Liver
Spleen
Lymph node
Thymus

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25
Q

Embryo

A

Mesoblastic phase

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26
Q

Fetal

A

Hepatic phase

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27
Q

Birth

A

Meduallry phase

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28
Q

Embry- Mesoblastic phase

A

2 weeks - 2 months gestation

Yolk sak - mesoderm layer

Hgb Formed
- Hgb Gower 1
- Gower 2
- Hgb Portland

Embryonyc Hgb^

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29
Q

Fetus - hepatic phase

A

2 months - 7 months

Liver and spleen

Hgb Formed:
- Fetal hemoglobin
- Hemoglobin A1 (small amount)

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30
Q

Birth- medullary phase

A

7 months - rest of life!

BM = intramedullary hematopoiesis - red marrow

Hgb Formed
- Hemoglobin A1
- Hemoglobin A2

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31
Q

Infancy and childhood

A

Volume and red marrow in infacts = red marrow in adults (distal long bones and axial skeleton)

at 4 years, yellow marrow, starts to replace red marrow –> limits sites of hematopoisis

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32
Q

active hematopoietic sites in axial skeleton

A

Sternum
Ribs
Pelvis
Vertebrae
Skull (after 40, no more skull)

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33
Q

Pelvis and sternum are

A

preferred bone marrow aspiration sites –> last place to be replaced by yellow marrow

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34
Q

Blood cell development theory

A

Monophyletic –> All blood cells arise from one precursor cell that is multipotential or pluripitenital and called stem cell

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35
Q

pluripotential/multipotential

A

can develop into any cell line in the body

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36
Q

progenitor cell

A

committed to cell line

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37
Q

cytokines

A

soluble messages to tell early cells to differentiate

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38
Q

Thrombopoietin

A

stimulates CFU-Meg, and causes release of platelets

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39
Q

Erythropoitetin

A

Stimulates CFU-E, and regulates erythroid progenitor cells to mature.

Glycorpotein prodcued by the peritubular interstitial cell in juxtaglomerular apparatus (kidney tubulus)

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40
Q

T cell

A

Cytotoxic, suppressing actvites

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41
Q

B cell

A

antibody production, can futher develop to plasma cell and secrete antibodies

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42
Q

CFU-GEMM

A

gives rise to a serius of committed cells (MYELOID)

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43
Q

BM

Myeloid: Eryrhoid ratio

A

2:1 - 5:1

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44
Q

Eythrpid maturation

A

Large –> small/pyknotic (condensation and shrinkage through degeneration)

Euchromatin –> heterochromatin –> Nucleus is extruded

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45
Q

Cell development: Asynchrony

A

Megaloblastic anemia

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46
Q

Basophilic Normoblast/prorubricyte

A

Chromatin: Some granularity
Cytoplasm: Slighlty less basophilic “Cornflower blue”

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47
Q

Polychromatophilic normoblast

A

Chromatin moderately compacted, condensed, “soccer ball”

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48
Q

Orhtochromatic normoblast (nRBC)

A

Last nucleated stage

Chromatin: completeley pyknotic

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49
Q

Polychromatiophilic erythrocyte

A

NO NUCLEUS

polychromatophilia

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50
Q

Wrights stain

A

polychromatiphilic erythrocyte

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51
Q

new methylene blue (vital stain)

A

reticulocyte

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52
Q

mature erythrocyte

A

central pallor

no longer able to synthesize hemoglobin

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53
Q

RBC membrane

A

selective barrier

Water and anions - passive diffusion

cations and others - active transport

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54
Q

potassium

A

found inside RBC 25:1

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55
Q

sodium is found

A

outside RBC 1:12

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56
Q

Outer hydrophilic portion of RBC membrane

A

Containts glycolipids, glycoproteins, proteins

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57
Q

central hydrophobic layer

A

contains proteins, cholesterol, phosphilipids

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58
Q

inner hydrophilic layer

A

protein

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59
Q

cholesterol

A

Central hydrophobic layer

  • Target cells
  • Acanthocytes
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60
Q

Choline phosphilipid

A

phosphatidyl choline and sphingomylein

outer half

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61
Q

amino phospholipids

A

phosphatidylethanolamine and phosphatidyl serine

inside

if outside, can initaite clotting

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62
Q

integral protein example

A

glycophorin

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63
Q

peripheral membrane pritein

A

spectrin, beneath lipid bilayer, forms cytoskeleton

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64
Q

Glycophorin

A

Principle RBC integral membrane protein

sialic acid

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65
Q

spectrin

A

most abundant peripheral membrane protein

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66
Q

RBC deformability

A

RBC survival through microvasculature
Oxygen deliver function

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67
Q

hgb infant

A

14-22g/dl

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68
Q

hgb male

A

14-18g/dl

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69
Q

hgb female

A

12-16g/dl

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70
Q

Hemoglobin =

A

4 heme rings
4 iron
4 globin chains

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71
Q

protophoryns

A

precursor of heme

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72
Q

1 heme ring =

A

1 porphoryin = 4 pyrrole rings

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73
Q

major rate limiting step in heme syntehsis

A

ALA synthetase

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74
Q

HgbA1

A

2a + 2 b

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75
Q

Hgb A2

A

2a+2d

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76
Q

HgbF

A

2a+2y

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77
Q

methemoglobin

A

Iron is oxidied to ferric state (Fe3+)

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78
Q

carboxyhemoglobin

A

Oxygen is replaced by CO - increased binding

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79
Q

sulfhemoglobin

A

sulfur incorporated into heme structure

irreversible

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80
Q

90% of RBC energy comes from

A

non-oxidative pathways

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81
Q

RES

A

Removes RBC from circulation
- spleen

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82
Q

spleen

A

primary site of phagocytosis

home to littoral cells

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83
Q

Extravascular hemolysis

A

Heme –> biliverdin –> unconjugated bilirubin –> conjugated bilirubin –> urobilinogen

Urobilinogen eliminated in stool

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84
Q

Hgb released in blood vessel

A

Circulates - Hemoglobinemia
- Filtered at the kidney = hemoglobinuria

Carried by haptoglobin to liver

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85
Q

if BM cannot keep up

A

Extramedullary hematopoiesis

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86
Q

renal failure patients

A

lack erythropoietic response to hypoxia

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87
Q

female RBC

A

4.2-5.4 x 10^6 ul

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88
Q

Male Rbc

A

4.7-6.1x10^6 ul

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89
Q

HCT

A

Proportion of red blood cells in whole blood

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90
Q

MCV

A

80-100fl

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91
Q

MCH

A

average Hgb mass/weight

27-31pg

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92
Q

MCHC

A

32-36 %

Average Hgb Concentration

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93
Q

neutrophil %

A

50-70%

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94
Q

lymphocyte %

A

20-44%

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95
Q

monocyte %

A

2-9%

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96
Q

band neutrophil

A

2-6%

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97
Q

eosinophil

A

0-4%

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98
Q

basophil

A

0-2%

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99
Q

Microcyte

A

diameter <6um
MCV<80

100
Q

normocyte

A

diam : 6-8um
MCV: 80-100fl

101
Q

anisocytosis

A

any significant variation in size

102
Q

macrocyte

A

diam: >9
MCV:> 100 fl

103
Q

microcyte cause

A

lack hemoglobin synthesis elements

104
Q

Macrocyte

A

impaired DNA synthesis

Megaloblastic Anemia (B12 deficiency)

105
Q

Myeloblast

A

no granules present

106
Q

Promyelocyte

A

Primary granules, CONTAINING Myeloperoxidase

107
Q

eosinophilic myelocyte

A

Filled with large red/orange secondary granules

108
Q

eosinophilic metabmyelocte

A

indention = less than half nuclear diameter

109
Q

Eosinophilic band

A

indention: >1/2 diameter

110
Q

Eosinophil - last stage

A

Bi lobed

Red orange secondary granules

111
Q

Basophilic myelocyte

A

filled with large purple/black secondary granules

112
Q

Dawn of neutrophilia

A

first distinguishable neutrophil

113
Q

Myelocyte –> metamyelocyte –>

A

band –> segmented

114
Q

primary granules

A

starts in promyelocyte stage

All granulocytes = non specific

115
Q

primary granules

A

specific for granulocytic line

116
Q

promonocyte

A

peaked crease

117
Q

tart cell

A

phagocytic cell engulfing at least the nucleus of another cell

118
Q

liver

A

kupffer cell

119
Q

lungs

A

alveolar macrophages

120
Q

Bone

A

osteoclasts

121
Q

skin and mucosa

A

langer hans

122
Q

Thrombopoiesis unique

A

cell volume increases

123
Q

megakaryoblast

A

Pseudopodia present

124
Q

promegakaryocyte

A

pseudopodia
fine azurophilic granules

125
Q

hyalomere

A

pale portion

126
Q

lymphoblast

A

intense staining at periphery

127
Q

Target cell

A

increased surface to volume ratio

128
Q

stomacyte

A

mouth like

BRC membrane cation permeability

129
Q

ovalocytes seen in

A

Megaloblastic anemia
IDA

130
Q

sickle cell (drepanocytes)

A

Hemoglobin S

131
Q

helmet cells (Bite cells)

A

“pitting”

132
Q

G6PD defieicny

A

Heinz bodies

133
Q

Howell jolly bodies

A

DNA remnants

134
Q

Basophilic stippling

A

RNA and mitochondrial remnants

135
Q

wright stain

A

pappenheimer bodies

136
Q

Prussian blue stain

A

sideritic granules

137
Q

rouleaux

A

stack of coins

lowering zeta potential - cells become sticky

138
Q

pelter huet anomaly

A

decreased lobulation

139
Q

Dohle Bodies

A

RNA remnants

140
Q

Auer Tods

A

Needle like bodies

fusion of primary granules

141
Q

Barr body

A

inactivated X chromosome

142
Q

microcytic, hypochromic anemia

A

Hgb synthesis defect

microcytic: <6 um
MCV<80

Hypochromic: CAP>30
MCHC<32

143
Q

Anemia definition

A

Deficiency of oxygen delivery to the tissues / healthy red blood cells

Low RBC count
Low hematocrit
Low hemoglobin

144
Q

Deficiency of iron

145
Q

Abnormal heme synthesis

A

porphoryias

146
Q

Abnormal globin chain synthesis

A

thalassemia

147
Q

Abnormal utilization of iron

A

Anemia of chronic disease
Sideroblastic anemia

148
Q

heme iron

A

complexed into a porphyrin ring to form heme ring of hemoglobin

149
Q

Transferrin

A

Carrier protein for serum iron

150
Q

Ferritin

A

Storage in BM and liver, easily mobilized

151
Q

Hemosiderin

A

Lysosomal membrane of macrophages, long term storage

152
Q

serum iron range

A

50-150 ug/dl

153
Q

TIBC

154
Q

Transferrin % saturation

155
Q

Serum ferritin male and female

A

M: 20-250
F : 10-120

156
Q

IDA cause - most common in western world

A

Blood loss
- mens: female
- GI bleed: male

157
Q

pica

A

abnormal cravings

158
Q

pagophagia

A

craving for ice

159
Q

geophagia

A

craving for dirt, clay

160
Q

koilinychia

A

spoon nails

161
Q

IDA lab findings

A

microcytic, hypochromic RBC

Ansisopoikilocytosis

162
Q

IDA

A

DECREASED SERUM FERRITIN –> found in all IDA stages

Increased TIBC

163
Q

Most common anemia found in hospitalized patients

A

ACD

Low serum iron, Normal or High Serum ferritin, Decreased TIBC

164
Q

ACD lab finding

A

N or Increased M:E ratio
due to decreased erythropoiesis

hemosiderin present

165
Q

ACD treatment

A

Corrrection of primary disease state

166
Q

SA lab findings

A

Dimorphic population
- Microcyte, normocyte
- hypochromic, normochromic

anisopoikiliocytosis

pappenheimer bodies

167
Q

SA LAB FINDINGS

A

Increased Serum ferittin
Normal or Low TIBC
Increased Serum Iron
Increased Transferrin saturation

168
Q

SA BM

A

Ringed sideroblast.
Erythroid hyperplasia

169
Q

hemochromatosis

A

tissue damage resulting from excess iron

170
Q

Heme synthesis issue

A

P8 deaminase

Prophobilinogen accumulation

171
Q

hemoglobinopatheies

A

qualitative or quantitative abnormalities in the synthesis of hemoglobin

172
Q

Thallasemia

A

Quantitative abnormalities resulting in decrease in production of globin chain synthesis

173
Q

definition of thalassemia

A

group of disorders that result in a decreased production of globin chains in hemoglobin usually resulting in a microcytic, hypochromic anemia

Alpha -> defect in alpha chain
beta –> defect in beta chain

174
Q

hemoglobin Bart

A

4 gamma chains

175
Q

Hemoglobin H

A

4 beta chains

176
Q

types of alpha thalassemias

A

Hemoglobin bart and H

177
Q

Alpha a thalassemia

178
Q

beta b thalassemia

A

ineffective erythropoiesis

179
Q

Beta (B) Thalassemia

A

Clinically seen 1st year of life

Forms HgbF and HgbA2

180
Q

B+

A

gene produces a reduced amount of beta chains

181
Q

BB

182
Q

Beta thalassemia minor

A

BB0,BB+ (trait)

183
Q

Beta thalassemia intermedia

A

B+B+ (less severe B+ types)

184
Q

B thalassemia Major

A

B0B0, B+B+. ,B0,B+

185
Q

B thalassemia major pathophysiology

A

bone changes (skull, long bones, hand)

Hepatospleenomeglay

186
Q

untreated B thalassemia major

A

Massive hepatosplenomegaly –> due to extramedaullar erythropoiesis

187
Q

treatment problems with B thalassemia major

A

iron overload

188
Q

B thalassemia major peripheral smear

A

Microcytic, hypochromic

Anisocytosis, poikilocytosis
Target cell

Howell jolly body

189
Q

alpha genes

190
Q

aa0, a+a+

A

a thalassemia minor

191
Q

Hemoglobin H disease

192
Q

Hemoglobin bart (4 gamma)

193
Q

Hemoglobin H disease

A

5-40% Hgb H (4 beta chains)

194
Q

Hemoglobin Bart

A

4 gamma chains

Lethl disease

4gamma chains

195
Q

Thalassemia findings

A

Microcytic, hypochromic
- reticulocytosis
- poikilocytosis - elliptocytes, target cells, fragments

LOW CBC

normal/high Iron

196
Q

Thalassemia, decreased osmotic fragility

A

due to higher surface to volume ratio

197
Q

Sickle cell

A

Hemoglobin S

A2B2 (6Glu-Val)

198
Q

beta 6th position

A

Glutamic acid –> valine

199
Q

SS anemia

A

Normocytic, Normochromci

Vasoocclusions –> Ischemic tissue injury

200
Q

SS - aplastic

A

Decreased reticulocyte count.

201
Q

SS - hemolytic

A

Increased Reticulocyte count

202
Q

SS- vaso-occlusive

A

occlusions of small blood vessels –> tissue damage and necrosis

203
Q

SS anemia - acute chest syndrome

A

2nd most common cause of hospital admission in SS

Occlusion in the pulmonary vasculature

204
Q

Dactylitis

A

painful swelling of hands and feet

205
Q

cutaneous manifestations

A

development of ulcers and sores, often on lower legs

206
Q

SS anemia, hand-foot syndrome

A

microinfraction in small bones, of hands and feet lead to unequal growthand bones feformities

Fingers and toes

207
Q

SS stroke

A

ischemia or hemorrhagic lesi0on in specific cerebral vessel

208
Q

hydroxyurea

A

increases Hgb F production

209
Q

Aplastic crisis, only one with

A

decreased reticulocyte count

  • sickled cells, (drepanocytes), oat cells
    Howell jollu
    Target cells
    Schistocytes
210
Q

Reduced HGb S is insoluble

A

turbid/opaque suspension

211
Q

Sickle Cell anemia

A

> 80% S
<20% Hgb F
TIny A2

212
Q

SS trait

A

60% Hgb A 1
40% Hgb S
Tiny A2
Tiny Hgb F

213
Q

Hemoglobin C

A

Glutamic acid –> lysine in 6th position

Hgb C crystals
95% Hgb C

trait: 40% hgb C

214
Q

Hemoglobin SC disease

A

Hgb SC crystals

50% Hgb S (valine)
50% Hgb C (lysine)

215
Q

Hemoglobin S disease

A

Microcytic, hypochromic

216
Q

GPI anchor (RBC membrane)

A

PNH (piga mutation)

217
Q

Hereditary stomacytosis

A

allowing Na+ into cell

218
Q

hereditary xertcytosis

A

leaking K+

219
Q

HPP

A

Spectrin (assembly issue)

Schisocytes

220
Q

HE

A

Elliptocytes (spectrin)

Defective/deficient

221
Q

Hereditary Spherocytosis

A

Spherocytes (spectrin), most common

Spectrin Deficiency
Loss of SA

222
Q

Hexose monophosphate pathway

A

GP6D deficiency

protect Hgb
Bite cells, Heinz bodies

223
Q

glycolytic pathway

A

Pyruvate kinase deficiency

non-oxidative

generates 90% of ATP needed by RRBC
Forms 2 ATP

low ATP –> Rigid RBC hemolysis

224
Q

Methemoglobin reductase pathway

A

methemoglobin reductase deficiency

important in maintaining heme iron in reduced (fe2_, ferrous form)

Methemoglobin
NADH reductase deficiency

225
Q

PNH lab finding

A

hemoglobinuria

226
Q

macrocytic anemia

227
Q

megaloblast

A

large and abnormal precursor cell in the BM exhibiting asynchrony

228
Q

Pancytopenia BM

A

Aplastic anemia

Congenital = fanconi
Aquired = idiopathic 2

229
Q

erythroid hyperplasia

A

congenital dyseryythropietic anemias

230
Q

erythroid hypoplasia

A

diamond BlackFan anemia

231
Q

Causes of impaired DNA synthesis (TTP synthesis inhibition)

A

Vitamin B12 deficiency
PERNICIOUS ANEMIA , lack of intrinsic factor

Folic acid deficiency

232
Q

IF

A

glycoprotein secreted by parietal cell (stomach)

233
Q

glossitis (b12 and folic acid )

A

smooth sore tongue

234
Q

B12 deficiency - 4 Ps

A

Peripheral Neuropathy
Pyramidal Tract signs
Posterior spinal column degradation
Psychosis

peripheral nerves are demyelinated

235
Q

Parestheia -=

A

tingling /prickling feeling

236
Q

aplastic anemia definition

A

hypoproliferative disorder with cellulae depletion and reduced production of all blood cells, pancytopenia

237
Q

cytopenia

A

abnormalitieis or deficiencies in specific blood cell elements

238
Q

pancytopenia

A

depression of each of the normal bone marrow elements

239
Q

Idiopathic

A

majority of cases of aplastic anemia

240
Q

Aplastic anemia - BM

241
Q

6, purple

A

EDTA, CBC, ESR

242
Q

1st choice for vein

A

median cubital vein

243
Q

antiseptic

A

any agent used to clean living tissue

244
Q

torniquet must

A

be removed before removing needle

245
Q

megaloblastic anemia peripheral smear

A

hypersegmentation
HJ bodies
Cabot Rings
Teardrop cell

246
Q

Megaloblastic anemia gives rise to Low serum folate

A

Folic acid deficiency

Low serum folate

247
Q

Magloblastic anemia VB12 deficiency

A

Low serum B12

Nuerological

Hematology (36 decks)

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