Hematology RBC Flashcards

1
Q

Pathognomonic in blood smear of G6PD

A

Heinz or Bite cells

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2
Q

AR due to chromosome breakage common in Ashkenazi Jews

Inc HBF and i Antigen

Hyperpigmentation
Short stature
Hypogonadism
Malformation of extremities
Microcephaly, mental retardation
Malformations of the heart and kidneys 

Pancytopenia after infancy and usually significant by 8 years

A

Fanconi Anemia

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3
Q
Microcephaly
DD
Cafe au lait spots
Bird-like facies
Micropthalmia
Ptosis
Strabismus, large ears
VSD
Horseshoe Kidney
Radial abnormalities
AR PANCYTOPENIA
A

Fanconi Anemia

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4
Q
Rare AR disorder
Microcephaly
Short stature
Abnormal facie
MR
PANCYTOPENIA
Hyperactivity, short attention span
A

Dubowitz Syndrome

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5
Q

Patients with inherited pancytopenia disorders have

A

chromosome fragility in metaphase preparations of PBL

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6
Q

Treatment for Acquired Pancytopenia

A

Antithymocyte Globulin

Cyclosporin

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7
Q
Rare congenital bone marrow failure, AD
Anemia, normo and macro
Reticulocytopenia
Absent RBC precursors in normal BM
Craniofacial abnormality 50%
Skeletal anomalies 
GU
Short stature
A

Congential Hypoplastic Anemia

Diamond-Blackfan Anemia

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8
Q

Diamnond Blackfan Anemia results from mutations jn

A

RPS19 gene of small 40s ribosome unit

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9
Q

Most common acquired red cell aplasia in children

A

Transient Erythroblastopenia of Childhood

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10
Q
Macrocytic anemia
Anisocytosis, Poikilocytosis
Dec retic count
Neutropenia
Hypersegmented neutrophils
INCREASED serum ldh
A

Folic Acid Deficiency Anemia

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11
Q

FA Deficiency Anemia tx

A

0.5-1.0mg per day PO or IV 3-4 weeks

Maintain at 0.2mg per day if with response

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12
Q
Weakness
Fatigue
Failure to thrive
Glossitis
Pallor
NEUROLOGIC SX
Macrocytic anemia
Inc urinary Methylmalonic Acid
A

Cobalamin Deficiency Anemia

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13
Q

Diagnostic for B12 deficiency

A

Methylmalonic acid

N 0-3.5mg/24

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14
Q

Tx Cobalamin Anemia

A

Vit B12 1.0mg parenteral oral will NOT work

Maintain with 1mg/day IM monthly

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15
Q

Reduction in circulating RBC mass

A

Anemia

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16
Q

Hypoxia presents with (4)

A

Weakness, fatigue, dyspnea
Pale conjunctiva skin
Headache, lightheadedness
Angina CAD

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17
Q

Markers to measure RBC mass (3)

A

Hb
Hct
RBC count

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18
Q

Anemia in males & females

A

<13.5 g/dl M

< 12.5 g/dl F

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19
Q

Microcytic anemia

A

MCV < 80

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20
Q

Normocytic

A

MCV 80-100

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21
Q

Macrocytic

A

MCV >100

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22
Q

Occur due to dec production of Hgb and extra division of cell

A

Microcytic anemia

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23
Q

Hemoglobin is composed of

Heme is composed of

A

Heme + Globin

Protoporphyrin + Fe

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24
Q

Inavailability of iron to be used for hemoglobin synthesis

A

Anemia of chronic disease

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25
Q

Any circumstance that dec production of protoporphyrin

A

Sideroblastic anemia

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26
Q

Microcytic anemia (4)

A

IDA
Anemia of chronic disease
Sideroblastic anemia
Thalassemia

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27
Q

Due to dec level of iron in the body, most common type of anemia

A

IDA

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28
Q

Most common nutritional deficiency in the world

A

Iron

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29
Q

Consumed in heme (meat derived) and nonheme (veg derived)

A

Iron

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30
Q

More readily absorbed form of iron

A

Heme form

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31
Q

Iron is absorbed in the

A

Duodenum

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32
Q

Transporter that Regulates uptake of iron to blood

A

Feroportin

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33
Q

Iron loss physiologically

A

Is not done by body

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34
Q

Decides whether iron should go into blood

A

Enterocyte

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35
Q

Iron has ability to that’s why it is always bound

A

Generate free radicals

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36
Q

transports iron and delivers it to liver and bone marrow macrophage for storage

A

Transferrin

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37
Q

Stored intracellular iron is bound to

A

Ferritin

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38
Q

For every 3 ferritin molecules,

carries iron

A

1

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39
Q

How much iron in blood

A

Serum iron

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40
Q

How many transferrin molecules in blood whether bound or not

A

Total Iron Binding Capacity

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41
Q

How many of transferrin molecules are bound by iron

A

Percent saturation

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42
Q

How many iron are in storage sites BM Macrophage and liver

A

Serum ferritin

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43
Q

Most common etiology of IDA (2)

A

dietary def

blood loss

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44
Q

In adults IDA occurs bec

A

PUD male

Menorrhagia or Pregnancy female

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45
Q

IDA in elderly bec of

A

Colon polyp/carcinoma

Hook worm

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46
Q

In neonate and infants IDA happens bec

A

Lack of iron in breastmilk

Poor diet

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47
Q

Hookworms that cause IDA

A

Nicator

Ancylostoma

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48
Q

Form of iron readily absorbed

A

Fe2 (ferrous) acidic form

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49
Q

Why can gastrectomy cause IDA

A

Bec gastric acid maintains acidic state fe2 hence if lost, not absorbed

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50
Q

Stages of Iron Def (4)

A

Storage iron depleted
Serum iron depleted
Normocytic anemia
Microcytic, hypochromic anemia

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51
Q

If ferritin goes down, TIBC

A

goes UP bec transferrin is released to look for Fe2 mol

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52
Q

Consumption of serum iron results in

A

Dec of percent saturation of iron

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53
Q

Normal percent saturation of iron

A

33%

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54
Q

Very early stage of IDA, rbcs are

before

A

Normocytic

Before becoming microcytic hypochromic

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55
Q

Clinical features of IDA (3)

A

Anemia
Koilonychia (spoon shaped cell)
Pica (chewing on dirt, seeking iron within dirt)

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56
Q
Microcytic, hypochromic anemia with inc RDW
Dec ferritin 
Inc TIBC
Dec serum iron and percent saturation
Inc FEP
A

IDA

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57
Q

RDW in IDA is inc because

A

Of wide spectrum of size of rbc

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58
Q

Test to check for IDA

A

Free erythrocyte protoporphyrin

increased

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59
Q

RBCs are about the size of

A

Lymphocyte nucleus

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60
Q

Tx of IDA

A

Ferrous sulfate

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61
Q

IDA
Esophageal web
Atrophic glossitis
Dysphagia

A

Plummer/Vinson Syndrome

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62
Q

Most common type of anemia in hospitalized patients associated with chronic inflammation of cancer

A

Anemia of chronic disease

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63
Q

Chronic disease cause IDA by secreting

A

acute phase reactant

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64
Q

Acute phase reactant that sequesters iron in storage sites

A

Hepcidin

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65
Q

Hepcidin causes IDA

A

By limiting transfer of iron from macrophage to erythroid precursors

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66
Q

Hepcidin also supresses

A

EPO

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67
Q

Body releases hepcidin to

A

hide iron from bacteria which is needed in division except not always bacterial

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68
Q

Decreased available iron leading to microcytic anemia

A

Anemia of chronic disease

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69
Q
Inc ferritin
Dec TIBC
Dec serum iron percent saturation
Inc FEP
Initially normocytic then
Microcytic
A

Anemia of chronic disease

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70
Q

Tx for ACD

A

exogenous EPO

treat underlying disease

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71
Q

Anemia due to defect protoporphyrin synthesis

Microcytic

A

Sideroblastic anemia

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72
Q

Final reaction of protoporphyrin synthesis is

Occurs in

A

Attachment of protoporphyrin to iron to make heme

Mitochondria

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73
Q

Rate limiting step in protoporphyrin synthesis

A

Succinyl coa synthesized to ALA by Aminolavulenic acid synthase

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74
Q

Cofactor for ALAS in protoporphyrin synthesis

A

B6 Pyridoxine

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75
Q

ALA is synthesized to porphobilinogen by

A

ALA dehydrogenase

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76
Q

Catalyzes joining of protoporphyrin with iron to become heme

A

Ferrochelatase

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77
Q

If protoporphyrin is deficient, what happens to iron

A

trapped in mitochondria

creating iron load around nucleus of cell

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78
Q

Iron laden mitochondria forming a ring around nucleus

A

Ringed sideroblast

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79
Q

In ring sideroblasts, iron is found in

A

mitochondria

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80
Q

Congential defect in sideroblastic anemia arises from

A

ALAS

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81
Q

Acquired causes of sideroblastic anemia (3)

A

Alcoholism
Lead poisoning
B6 Pyridoxine deficiency

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82
Q

Alcoholism causes sideroblastic anemia

A

bec it is a mitochondrial poison

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83
Q

Lead poisoning causes

A

Denaturation of ALAD and Ferrochelatase

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84
Q

Denaturation of ALAD and Ferrochelatase leads to

A

Dec production of protoporphyrin iron trapping in mitchondria

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85
Q

B6 Pyridoxine deficiency causes sideroblastic anemia bec

A

B6 is a cofactor of ALAS the rate limiting step for production of protoporphyrin

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86
Q

Vitamin B6 deficiency is common in patients

A

taking isoniazed hence sideroblastic anemia

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87
Q

Inc ferritin
Dec TIBC
Inc serum iron and percent saturation

A

Sideroblastic anemia

88
Q

Sideroblastic anemia has inc serum iron bec

A

Rupture of iron loaded rbc leading to ingestion of macrophage of iron

89
Q

Iron overloaded state

A

Hemochromatosis

90
Q

Due to dec synthesis of globin chain of hb

A

Thalassemia

91
Q

Dec globin lead to dec Hb resulting in

A

microcytic anemia

92
Q

Defective globin chain

A

Sickle cell anemia

93
Q

Carriers of thalassemia are protected against

A

Plasmodium falciparum

94
Q

Dec production of alpha or beta globin chain

A

Thalassemia

95
Q

A2Y2

A

HbF

96
Q

A2B2

A

HbA

97
Q

A2D2

A

HbA2

98
Q

Gene deletion

Normally 4 alpha alleles on chromosome 16

A

alpha thalassemia

99
Q

1 alpha gene deleted

A

asymptomatic

100
Q

2 alpha genes deleted

A

Mild anemia with slightly inc RBC count

101
Q

Worse deletion of gene

A

Cis more trans bec of inc risk of severe thalassemia in offspring
Asians

102
Q

3 genes deleted

A

severe anemia

B chain forms tetramers that damage rbc

103
Q

4 genes deleted

A

lethal in utero hydrops fetalis

Y chain forms tetramers that damage RBCs

104
Q

Tetramer of gamma chains

A

Hemoglobin bart

105
Q

Two beta genes present on

A

Ch 11

106
Q

Gene mutation in absent beta or diminished production of B globin chain

A

B thalassemia

107
Q

Mutation resulting in absent beta chain

A

Beta nau

108
Q

Diminished production of b globin chain

A

Beta plus

109
Q

Mildest form of beta thalassemia

A

Beta thalasemia minor B normal and B plus

Asymptomatic

110
Q

Microcytic hypochromic

Target cells

A

Beta thalassema minor

111
Q

Bleb of membrane protruding at central portion of rbc creating redness instead of pallor

A

target cell

112
Q

Dec cytoplasm

Inc amount of membrane

A

Target cell

113
Q

Slightly dec HbA
Inc HbA2 to 5%
Inc HbF 2%

A

Beta thalassemia minor

114
Q

Most severe form
B nau beta nau
Severe anemia few months after birth
HbF temporarily protective

A

B thalassemia major

115
Q

Ineffective erythropoiesis

Extravascular hemolysis bec of

A

A tetramer aggregation

116
Q

Expansion of hematopoiesis into marrow of skull and facial bones

Extramedullary hematopoiesis with HSN

Risk of aplastic crisis with ParvoB19

A

Massive erythroid hyperplasia

117
Q

Expansion of hematopoiesis into marrow of skull and facial bone result in

A

Crew cut appearance on xray

Chipmunk like face

118
Q

Tx thalassemia

A

Chronic transfusion

119
Q

Transfusion leads to inc risk of

A

Secondary hemochromatosis

120
Q

Microcytic
Hypochromic
Target cells
Nucleated RBC

A

Beta thalassemia major

121
Q

No HbA

A

Beta thalassemia major

122
Q

macrocytic anemias are most commonly due to

A

Vit B12 and folate deficiency

123
Q

Macrocytic anemia with less division of cell due to loss of precursor

A

> 100 MCV

124
Q

Tetrahydrofolated undergoes

to participate in synthesis of DNA precursor

A

Methylated to enter then gives methyl to VB12

125
Q

Vit B12 passes methyl to

A

Homocysteine

Becoming methionine

126
Q

Important in transfer of methyl groups to other molecules

A

Methionine

127
Q

Deficiency of this nutrition will not permit DNA precursor formation

A

Folate / Tetrahydrofolate

128
Q

Folate and B12 def (3)

A

Megaloblastic anemia
Hypersegmented neutrophils >5 lobes
Megaloblastic change

129
Q

Other causes of macrocytic anemia (3)

A

Alcoholism
Liver disease
Drugs (5-FU)

130
Q

Difference between macrocytic anemia vs megaloblastic

A

Large RBCs without hypersegmented neutrophil and no megaloblastic change in rapidly dividing cells in mictocytic anemia

131
Q

Folate is absorbed in

A

jejunum

132
Q

Folate deficiency develops within

A

months

133
Q

Methotrexate causes megaloblastic anemia by

A

inhibiting dihydrofolate reductase

134
Q
Macrocytic rbc hypersegmented neutrophil
Glossitis 
Dec serum folate
INC serum homocysteine
Normal methylmalonic acid
A

Folate acid def megaloblastic anemia

135
Q

Inc in homocysteine in folic acid def is due to

A

lack of transfer of methyl group by B12 to homocysteine inc methionine

136
Q

Gets converted to succinyl coa via B12 pyridoxine

A

Methylmalonic acid

137
Q

Differentiates folic from b12 pyridoxine megalobastic anemia

A

Methylmalonic acid

Normal in folic acid

138
Q

Vit B12 is bound to a protein produced by salivafy gland

A

R-binder

139
Q

B12 is absorbed in

A

Ileum

140
Q

Binds B12 secreted by parietal cell

A

Intrinsic factor

141
Q

Less common and years to develop bec of large hepatic storage

A

B12 pyridoxine deficiency

142
Q

Most common cause of B12 deficiency from autoimmune destruction of parietal cells leading to IF deficiency

A

Pernicious anemia

143
Q

Other causes of VitB12 (3)

A
Pancreatic insufficiency (produces enzyme that cleaves b12 fr R-binder)
Damage to terminal ileum by D latum or Crohn’s
Dietary deficiency
144
Q

Macrocytic anemia hypersegmented neutrophils
Glossitis
Subacute combined SPINAL CORD DEGENERATION
Neurologic symptoms
Dec serum b12
Inc serum homocysteine
Inc methylmalonic acid

A

Vit B12 Pyridoxine deficiency

145
Q

Vit B12 is involved in (2)

A

Synthesis of DNA precursors

Transfer of methyl (conversion of methylmalonic acid to succinyl coA) inc succinyl coa in SC causes degeneration

146
Q

Normocytic anemia due to (2)

A

Inc peripheral destruction

Underproduction

147
Q

Helps distinguish etiology of normocytic anemia

A

Retic count

148
Q

Young rbcs released from bm larger with bluish cytoplasm

A

Reticulocytes

149
Q

Bluish hue in cytoplasm is bec of

A

RNA

150
Q

Normal retic count

A

1-2%

151
Q

Marrow responds to anemia by

A

Increasing retic count to more than 3

152
Q

Retic count in anemia is falsely elevated bec

A

it is percentage of total blood cell

Dec in total rbc falsely elevates retic count

153
Q

Correction of retic count

A

Multiply RC by Hct/45

154
Q

> 3% RC suggests

<3% suggests

A

Functioning marrow

Underproduction by marrow

155
Q

Anemia with good marrow response may be intra or extra

A

Peripheral RBC hemolysis

156
Q

Extravascular hemolysis occurs in

A

RES spleen liver lymph mac

157
Q

Intravascular hemolysis occurs in

A

vessels

158
Q

Extravascular hemolysis findings (4)

A

Anemia with splenomegaly
Jaundice by unconjugated bilirubin
Inc bilirubin gallstone
Marrow hyperplasia with corrected RC of >3%

159
Q

Inc hemoglobin in blood due to destruction of rbc within vessels

A

Intravascular hemolysis

160
Q

Hemoglobin in blood is bound to

A

Haptoglobin and eventually lost in serum

161
Q

Hemoglobinuria occurs

A

Due to build up of hemoglobin in the blood (hemoglobinemia)

162
Q

When hemoglobin is taken up by renal tubular cells, destroyed and piled up

A

Hemosiderinuria days later

163
Q

Hemoglobinemia
Hemoglobinuria
Hemosiderinuria
Dec serum haptoglobin

A

Intravascular hemolysis

164
Q

Defect in myeloid stem cell resulting in absent GPI acquired rendering cell suceptible to complement destruction

A

Paroxysmal nocturnal hemoglobinuria

165
Q

Protects rbcs from complement system (2)

A

Decay accelerating factor decays C3 convertase

Membrane inhibitor of reactive lysis

166
Q

DAF and MIRL is connected to rbc by

A

By GPI ankrin protein

167
Q

Shallow breathing at night with inc CO2 leads to complement activation by

A

acidosis

168
Q

Hemoglobinuria upon waking up due to episodic intravascular hemolysis

A

PNH

169
Q

Hemoglobinemia
Hemoglobinuria upon waking
Hemosiderinuria days after hemolysis

A

PNH

170
Q

Screening test for PNH

A

Sucrose to activate complement

171
Q

Confirmtatory test for PNH

A

Acidified serum test activate complement

Flow cytometry to detect lack CD55 (DAF)

172
Q

Main cause of death in PNH

A

thrombosis via activation of coagulation cascase of platelets

173
Q

Complications of PNH

A

IDA (loss of Hbg loss of iron)

10% in AML

174
Q

X linked recessive disorder rendering cell susceptible to oxidative stress

A

G6PD Deficiency

175
Q

G6PD produces to regenerate glutathione from GSSG

A

NADPH

176
Q

Two variants of G6PD

A

African

Mediterranean

177
Q

Mildly reduced half life of G6PD
Loss of G6PD in older cells
Lysis of older cell

A

African variant

178
Q

Markedly reduced half life of G6PD

Earlier reduction of G6PD and hemolysis

A

Mediterranean

179
Q

Oxidative stresses that precipitate Hb (3)

A

Infection,
drugs (primaquine, sulfa, dapsone)
fava beans

180
Q

Precipitation of hg results in formation of

A

Heinz bodies

181
Q

Heinz bodies (hg precipitation) removed by splenic mac result in

A

bite cells

182
Q
Hemoglobinuria
Back pain (nephrotoxic) hours after exposure to oxidative stress
A

G6PD deficiency

183
Q

Used to screen for G6PD

Highlights precipitated hemoglobin

A

Heinz preparation

184
Q

Enzyme studies are done for confirmation

A

after acute disease

185
Q

Antibody mediated IgG IgM destruction of RBC

A

Immune hemolytic anemia

186
Q

Involves extravascula hemolysis

Binds rbc in warm central body

A

IgG mediated

187
Q

Consumption of splenic mac by membrane antibody coated RBC results in

A

spherocyte formation

188
Q

IgG mediated is associated with (3)

A

SLE
Chronic lymphocytic anemia
Drugs (attaches to rbc like penicillin and antibody binds drug mem complex to remove membrane)
induce production of autoantibodies like methyldopa binding antigen in rbc

189
Q

Tx of autoimmune hemolytic anemia

A

Cessation of drug
Steroid
IVIG -eat the injected instead of rbc buy time
Splenectomy to remove source of antibody and destruction

190
Q

Intravascular hemolysis occuring in colder extremities (cold agglutinib)

A

IgM mediated autoimmune hemolytic disease

191
Q

IgM mediated autoimmune is related to

A

Mycoplasma pneumoniae

Infectious mononucleosis

192
Q

Used to diagnose immune hemolytic anemia

A

Coombs test

193
Q

Confirms the presence of antibody coated RBC

A

Direct coombs test

Introduction of antibody against IgG to rbc
Agglutination occurs if RBCs are coated with IgG antibody

194
Q

Most important test for IHA

A

Direct coombs test

195
Q

Confirms present of antibody in serum

A

Indirect coombs

Anti IgG and test RBCs mixed with patient serum

Agglutination occurs if serum antibodies are present

196
Q

Intravascular hemolysis from microvascular pathology

RBCs destroyed as they are passed through circulation

A

Microangiopathic hemolytic anemia

197
Q

Develops when RBCs are sheared against platelet outgrowth in microvasculature

A

Schistocyte

Helmet cell

198
Q

platelet thrombi from lack of ADAMS TS13

A

TTP

199
Q

platelet microthrombi from toxin by ECOLI O15H7

A

HUS

200
Q

Platelet plus fibrin thrombi

A

DIC

201
Q

pregnant microangiopathic anemia in liver

A

HELLP

202
Q

Microangiopathic hemolytic occurs in (3)

A

Microthrombi
Prosthetic valves
Aortic stenosis

203
Q

Infection if RBC and liver with plasmodium by anopheles

A

Malaria

204
Q

Hemolysis occurs in malaria bec of

A

rupture of cell during life cycle of plasmodium

205
Q

Dec production of rbc with low reticulocyte count

A

Underproduction anemia

206
Q

Causes of underproduction anemia (3)

A

1) causes of micro and macrocytic anemia
2) renal failure
3) damage to bone marrow precursor

207
Q

Infects progenitor red cells stops EPO leading to anemia in marrow stress

A

Parvovirus B19

208
Q

Damage to HSC

Pancytopenia

A

Aplastic anemia

209
Q

Aplastic anemia pathognomonic

A

Empty marrow replaced with fat

210
Q

Aplastic anemia tx (5)

A
Cessation of drugs
Transfusion
Marrow stimulating factor (EPO, GM-CSF, G-CSF)
Immunosupression
BMT
211
Q

Pathologic process replacing bone marrow impairing hematopoiesis
Pancytopenia

A

Myelophthisic process

212
Q

CML must be distinguished from

A

Leukemoid reaction

213
Q

CML are (3)

A

1) granulocytes are LAP negative (leokocyte alkaline phosphatase for inflammation)
2) associated with inc basophil
3) exhibit t 9;22

214
Q

Neoplaatic proliferation of mature myeloid cell esp rbc

All increased

A

Polycythemia vera

215
Q

Polycythemia vera is driven by

A

JAK 2 KINASE MUTATION

216
Q
Blurry vision headache
Inc risk of venous thrombosis
Flushed face due to congestion
Itch after bathing
Dec EPO
A

Polycythemia vera

217
Q

Chronic, moderate to severe anemia usually manifest in infancy
Associated with normal WBC and platelets
Pure red cell aplasia as a result selective destruction of red cells in the bone marrow
Associated with increased Hemoglobin F (5-25%) and increased red cell adenosine deaminase ADA

Severe normocytic normochromic anemia
Bone marrow red cell aplasia, normal megakaryocytic and granulocytic cell lines

A

Diamond Blackfan Syndrome