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Hematology > Hematology Week 1: Intrinsic Hemolytic Anemias > Flashcards

Hematology Week 1: Intrinsic Hemolytic Anemias Flashcards

1
Q

Hemolytic Anemia Definition

A

Destruction of the red blood cells

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2
Q

Intrinsic Hemolytic Anemia Definition

A

Destruction of the red blood cells due to inherited cell structure/function defect

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3
Q

Intravascular hemolysis properties

A
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4
Q

Extravascular hemolysis properties

A
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5
Q

Intravascular vs Extravascular hemolysis properties

A
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6
Q

Intravascular hemolysis Key cell seen

A

Schistocytes (RBC Fragments)

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7
Q

Extravascular hemolysis Key Cell seen

A

Spherocytes

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8
Q

Intravascular hemolysis Haptoglobin

A

Decreased

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9
Q

Intravascular hemolysis Hemoglobinuria

A

Yes Hemoglobinuria

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10
Q

Intravascular hemolysis Comb’s Test

A

Negative DAT

Except for in PCH with MAC activation

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11
Q

Intravascular hemolysis example

A
  • Microangiopathic hemolytic anemia
  • mechanic hemolysis
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12
Q

Intravascular hemolysis etiology

A

Destruction of the RBCs within circulation through shredding of RBCs through thrombotic vessel or lyse of RBCs through complement activation

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13
Q

Extravascular hemolysis Etiology

A

Removal of a portion of the RBCs membrane by macrophages in spleen or liver due to surface antigen-antibody (Ag-Ab) complex or abnormal RBCs

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14
Q

Extravascular hemolysis Key Cell seen

A

Spherocytes

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15
Q

Extravascular hemolysis Hemoglobinuria

A

Negative

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16
Q

Extravascular hemolysis Hemosiderinuria

A

Negative

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17
Q

Extravascular hemolysis Comb’s test

A

Positive (IgG, C3)

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18
Q

Extravascular Hemolysis Examples

A

Immune-mediated hemolytic anemia

subset medication induced hemolytic anemia

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19
Q

Haptoglobin Function

A

Binds to free hemoglobin to prevent toxicity (intravascular hemolysis)

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20
Q

Site of Extravascular Hemolysis

A

Liver or Spleen

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21
Q

Intravascular hemolysis diagram

A

RBC going through thrombi shredding

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22
Q

Extravascular Hemolysis Etiology Diagram

A
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23
Q

Intravascular Hemolysis some lab values

A
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24
Q

Extravascular Hemolysis Some Lab Values

A
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25
Q

Common Clinical Findings of hemolytic Anemias

A

Pigment stone usually black in color

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26
Q

Common Pathology Findings in Hemolytic Anemia

A
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27
Q

How long does response to EPO take?

A

~7 days

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28
Q

Erythroid hyperplasia

A
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29
Q

Intrinsic Hereditary RBC abnormalities

3 listed

A
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30
Q

RBC Membrane defects examples

3 listed

A
  • Hereditary spherocytosis
  • Hereditary elliptocytosis
  • Hereditary pyropoikilocytosis
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31
Q

Normal RBC membrane

A
  • ankyrin complex maintain shape and deformability of RBC
  • when RBCs are traveling through the splenic sinuses they must have deformability
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32
Q

Pathogenesis of Spherocytosis

A

unstable membrane structure creates small vesicles that detach eventually forming a spherocyte

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33
Q

PAthogenesis of spherocytosis: Skeletal proteins?

4 listed

A
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34
Q

Hereditary spherocytosis blood smear features

3 listed

A
35
Q

First clue of hereditary spherocytosis

A

Family Hx

36
Q

How to test for Hereditary Spherocytosis

A

Osmotic Fragility test

37
Q

Osmotic Fragility Test

A

curve is wrong normal is dotted

mild is green

Hs is purple

38
Q

Hereditary Spherocytosis Caveats

4 listed

A
39
Q

RBC enzyme Deficiency G6PD

A
40
Q

Bite Cell… Think?

A

G6PD

41
Q

Glucose-6-phosphate Dehydrogenase Deficiency Pathogenesis

A
  • G6PD is critical in protecting cells from oxidant injury
  • G-6PD is critical in converting NADP to NADPH which then converts glutathione to reduced form GSH
  • Mature RBCs cannot make new G6PD because they lack a nucleus
  • When there is not enough G6PD the RBCs cannot protect themselves from oxidative injury
42
Q

G6PD deficiency Triggers

3 listed

A

Infection - most common

Drugs - antimalarial

Food - fava beans

43
Q

G6PD deficiency timeline

A

Hemolytic anemia develops 2-3 days after exposure to oxidative stress

Often self-limited because old cells will be deficient not the new cells

with spontaneous resolution in most patients

44
Q

G6PD deficiency Diagnosis

A

Serum G6PD levels after acute hemolysis is over

45
Q

When to test for G6PD deficiency?

A

after the acute hemolysis is over

46
Q

G6PD Deficincy Histological findings

A

Oxidative injury causes hemoglobin percipitates which are denatured globin which binds to the membrane and causes membrane damage called Heinz bodies

This can occur in intravascular and extravascular hemolysis

47
Q

How Bite Cells Form

A
48
Q

Variants of G6PD Deficiency

A

Important variants

G6PD A Moderate hemolysis

G6PDMED SEVERE

49
Q

G6PD Mutation

A

X linked

50
Q

Hemoglobinopathies

A
51
Q

Sickle Cell Disease AKA

A

Hemoglobin S

52
Q

Most common hemoglobinopathy

A

sickle Cell disease

53
Q

Sickle Cell disease Mutation

A

On one β chain Sickle Cell Trait

On both β Chain Sickle Cell Disease

54
Q

Sickle Cell confers resistance to?

A

Malaria infection

55
Q

SIckle Cell Disease Etiology

A

polymerization of Hb causing long sickle cells

56
Q

Sickling occurs with

A
  • Dehydration
  • Deoxygenation
  • High Altitude
57
Q

Repeated sickling of cells causes?

A

Permanent damage

58
Q

Sickle Cell Disease can cause

A
59
Q

Sickle Cell Screen Test

A

Sickle Cell Screen Test

Doesn’t distinguish sickle cell trait from sickle cell disease

60
Q

Sickle Cell Diagnosis

A
61
Q

Thalassemia Subtypes

A

α 4 genes

or

β 2 genes

62
Q

β Thalassemia Mutations and effect

A

splice sites and promoter of the β gene

63
Q

β Thalassemia Minor (trait)

A
64
Q

β Thalassemia Minor HbA2

A

Increased >3.5% on electrophoresis

65
Q

β Thalassemia Minor CBC values

A

mild anemia (hypochromic microcytic) or no anemia

MCV decreased

increased RBC

RDW is normal

66
Q

β Thalassemia Minor Symptoms

A

Usually asymptomatic

67
Q

How to differentiate between Iron Deficiency Anemia and β Thalassemia Minor from CBC

A

IDA RBC low RDW High

Thal RBC High RDW Norm

68
Q

β Thalassemia Major

A

Cannot make any β Hb Chain

69
Q

β Thalassemia Major things to remember

5 listed

A

Require Transfusion

Marrow Expansion

Extramedullary hematopoiesis

Risk of Parvovirus B19 induced aplastic crisis

Severe Anemia

70
Q

β Thalassemia Major increased risk of?

A

β Thalassemia Risk of Parvovirus B19 induced aplastic crisis

71
Q

Why is β Thalassemia Major damaging to the body

A

excess α chains clump together and cause damage to erythroid precursors

72
Q

β Thalassemia Major major effects

7 listed

A
  • Reduced RBC production and survival
  • Ineffective erythropoiesis
  • Systemic iron overload
  • Desctruction of spleen
  • skeletal abnormalities
  • Need stem cell transplant
  • Severe Anemia
73
Q

β Thalassemia Major treatment

4 listed

A
74
Q

α Thalassemia # of genes

A

2 genes with 2 copies so 4 copies total

allowing some loss

75
Q

spectrum of α Thalassemia

A
76
Q

Deletion of 1 copy of α Thalassemia

A

silent carrier

77
Q

α Thalassemia Deletion of 2 copies

A

α Thalassemia traint (+/-) anemia

78
Q

α Thalassemia Deletion of 3 copies

A

Severe anemia

β tetramers (HbH Disease)

79
Q

α Thalassemia Deletion of 4 copies

A

Hydrops Fetalis

Lethal in utero

80
Q

HbH Disease

A
  • H bodies (soluble bodies of β Globin subunits) are minimally damaging to RBCs
  • not nearly as bad as β Thalassemia Major
81
Q

Epidemiology of Malaria vs G6PD Deficiency

A

Severe hemolysis protects against Plasmodium falciparum malaria

82
Q

Which is worse β or α Thalassemia?

A

β because β tetramers aren’t as damaging in α thalassemia

83
Q

Insert Summary Review slides

A

Hematology (26 decks)

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