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Hematology > Hematology Week 1: Extrinsic Hemolytic Anemias > Flashcards

Hematology Week 1: Extrinsic Hemolytic Anemias Flashcards

1
Q

Extrinsic Hemolytic Anemias Definition

A

Destruction of RBCs from extracellular etiology

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2
Q

Major types of Extrinsic Hemolysis

A
  • Immune-mediated hemolytic anemia (IHA)
  • Microangiopathic Hemolytic Anemia (MAHA)
  • Mechanical damage
  • Drug-induced hemolytic anemia
  • Malaria-induced hemolysis
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3
Q

WarmAIHA DAT Test

A

IgG

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4
Q

ColdAIHA DAT Test

A

IgM, C3

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5
Q

PCH DAT Test

A

Biphasic polyclonal IgG

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6
Q

WAIHA Etiology

3 listed

A
  • 50% Idiopathic
  • LPD
  • Autoimmune Diseases
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7
Q

WAIHA T of Ab binding

A

37*C

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8
Q

WAIHA T of Hemolysis

A

37*C

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9
Q

WAIHA Site of Hemolysis

A
  • Extravascular
  • Spleen macrophages
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10
Q

WAIHA Treatment

A

Glucocorticoids 1*

Splenectomy 2*

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11
Q

CAIHA Etiology

A
  • LPD (CLL)
  • Infections (Mycoplasma pneumonia, EBV, HIV)
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12
Q

CAIHA T of Ab binding

A

1-5*C

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13
Q

CAIHA T of Hemolysis

A

37*C

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14
Q

CAIHA Site of hemolysis

A
  • Extravascular
  • Liver
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15
Q

CAIHA Treatment

3 listed

A
  • Avoid Cold
  • Supportive
  • Treat underlying disease
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16
Q

PCH Etiology

A
  • Upper respiratory infection (children
  • Aggressive Lymphoma (rare, adults)
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17
Q

PCH T of Ab binding

A

Cold

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18
Q

PCH T of Hemolysis

A

37*C

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19
Q

PCH Site of Hemolysis

A

Intravascular Sudden and Massive

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20
Q

PCH Treatment

A

Supportive care

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21
Q

MAHA AKA

A

Microangiopathic Hemolytic Anemia

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22
Q

Types of MAHA

3 listed

A
  • Disseminated Intravascular Coagulation (DIC)
  • Thrombotic Thrombocytopenic Purpura (TTP)
  • Hemolytic Uremic Syndrome (HUS)
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23
Q

IHA AKA

A

Immune-mediated Hemolytic Anemia

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24
Q

Immune-mediated Hemolytic Anemia

A

destruction of circulating RBCs in the setting of anti-RBC antibodies against self-antigens on the RBC surface

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25
Q

DAT AKA

A

Direct antiglobulin test

or

Coombs test

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26
Q

Blood Findings of IHA

4 listed

A
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27
Q

Spherocytes seen in?

2 listed

A
  • IHA
  • Hereditary spherocytosis
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28
Q

WAIHA Overview

A
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29
Q

Etiology of WAIHA

A
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30
Q

most common tupe of IHA

A

WAIHA

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31
Q

1/3 of SLE patients have?

A

WAIHA

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32
Q

Pathogenesis of WAIHA

A
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33
Q

WAIHA Extravascular or Intravascular?

A

Extravascular

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34
Q

PB Smear in WAIHA

A
  • multiple spherocytes
  • Spherocytes do not have central pallor
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35
Q

WAIHA Clinical Presentations

4 listed

A
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36
Q

Treatment of WAIHA

6 listed

A
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37
Q

Definition of CAIHA

A
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38
Q

CAIHA Intravascular or Extravascular

A

Extravascular hemolysis in the liver

and can occur intravascularly!

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39
Q

Etiology CAIHA

A
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40
Q

Common causes of CAIHA

A
  • Chronic Lymphocytic Leukemia (CLL)
  • Mycoplasma Pneumonia
  • EBV and HIV
  • SLE
  • Idiopathy
  • Cancer
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41
Q

Clinical Manifestations of Cold Agglutination Disease CAIHA

A
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42
Q

CAIHA AKA

A

Cold Agglutination Disease

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43
Q

Unique Laboratory findings in CAIHA

4 listed

44
Q

Special test for CAIHA

A

Cold agglutinin titer will be elevated in CAIHA

45
Q

PB smear in CAIHA

A

clumps of RBCs from IgM linking

46
Q

Treatment of CAIHA

47
Q

Most common cause of CAIHA

48
Q

PCH AKA

A

Paroxysmal Cold Hemoglobinuria

49
Q

PCH Etiology

A
  • most frequently seen in childer with antecedent upper respiratory viral, mycoplasma, bacteria infection or immunization
  • secondary to untreated tertiary syphilis but is exceedingly rare
  • rarely secondary to aggressive lymphomas
50
Q

PCH Pathogenesis

4 listed

A
  • Infection triggers formation of polyclonal biphasic IgG (Donath-Landsteiner antibody) that corss react with P antigen on the RBC membrane
  • IgG attaches to RBCs in cold temperatures in the extremities forming P-anti-P antigen-antibody complexes which are potent activators of complement
  • When the complex returns to a warmer central circulation the complement system activates the MAC and causes intravascular hemolysis
  • Usually a sudden onset with massive hemolysis
51
Q

Donath-Landsteiner Antibody

52
Q

Clinical presentation of PCH

53
Q

Blood fings in PCH

A

neutrophils phagocytosing RBCs is characteristic

54
Q

Unique Lab test for PCH

A

Donath Landsteiner Test

55
Q

Unique Lab findings in PCH

56
Q

Drug-induced hemolytic anemia

57
Q

Treatment of PCH

58
Q

Most common drug causing drug-induced hemolytic anemia

A

Cephalosporin

59
Q

Drug-induced hemolytic anemia subsets

60
Q

Drug-induced hemolytic anemia subsets most common?

A

penicillins/cephalosporin

61
Q

Specific test for Drug-induced hemolytic anemia

62
Q

Lab findings of Drug-induced Hemolytic Anemia

63
Q

Comparison of WAIHA, CAIHA PCH

A

cold is extra and intravascular!!!!!!

64
Q

Question 1

A

E Microspherocytes

65
Q

when do you see macroovalocytes?

A

in foltae and B12 difficiency anemia

66
Q

When do you see hypochromic microcytic cells?

A

Iron Deficiency Anemia

67
Q

When do you see polychromatic cells?

A

Any time a patient has anemia except for Iron Deficiency Anemia

68
Q

When do you see Schistocytes?

A

intravascular hemolysis

69
Q

When do you see microspherocytes?

A

Immune-mediated hemolytic Anemias

70
Q

Question 2

A

B Idiopathic

71
Q

Question 3

A

D Extravascular spleen will see splenomegaly

72
Q

MAHA AKA

A

Microangiopathic hemolytic anemia

73
Q

MAHA characteristic blood finding

A

schistocytes

74
Q

Major Types of MAHA

6 listed

A

most common are?

Disseminated intravascular coagulation (DIC)

Hemolytic Uremic Syndrome (HUS)

Thrombotic Thrombocytopenic Purpura (TTP)

75
Q

2 common pathways of intravascular hemolysis

76
Q

Lab findings in MAHA

8 listed

77
Q

DIC AKA

A

Disseminated Intravascular Coagulopathy

78
Q

DIC Etiology

A

DIC IS A MEDICAL EMERGENCY

79
Q

DIC Pathogenesis

80
Q

Lab findings in DIC

4 listed

81
Q

D-dimer in DIC

A

Significantly increased

82
Q

Fibrinogen in DIC

A

Decreased fibrinogen level

83
Q

TTP AKA

A

Thrombotic thrombocytopenic Pupura (TTP)

84
Q

TTP Definition

A

Systemic aggregation of platelets within the vasculature generally in arteries and arterioles causing microvascular thrombosis, hemolytic anemia and thrombocytopenia

85
Q

Hereditary TTP

A

Mutations of ADAMTS13 genes

Markedly decreased ADAMTS13 level

86
Q

Acquired TTP

A

Autoimmune phenomenon associated with malignancy, HSCT, pregnancy, medications or infection such as HIV

Markedly decreased ADAMTS13 level

Anti ADAMTS13 antibody

87
Q

Commonality of Hereditary TTP and Acquired TTP

A

Markedly decreased levels of ADAMTS13 levels

88
Q

Function of Von Willebrand Factor

A

ADAMTS13 cuts vWF

when deficient causes coagulation

89
Q

TTP Pathogenesis

A

extra von Willebrand factor (vWF)

90
Q

Mortality in TTP

A

90% mortality if untreated

91
Q

Plasmic score

A

indivative of TTP

92
Q

HUS AKA

A

Hemolytic Uremic Syndrome

93
Q

Typical HUS

A

Shiga-like toxin-associated HUS (Stx-HUS)

infections with E. coli in 75% of cases

Enterococcus or Shigella in some cases

94
Q

Atypical HUS

A
  • Sporadic or complement-mediated
  • up to 65% of patients have mutations in genes coding for either
  • inactivation mutations of complement regulatory proteins (factor H, factor I, membrane cofactor protein (MCP or CD46)
  • Activation mutations of components of the alternative C3 convertase (Factor B, C3)
  • Result in constitutively activated alternative complement pathway
95
Q

Comparison of DIC, TTP and HUS

96
Q

Clinical Features of HUS

5 listed

97
Q

Question 5

A

schistocytes in the peripheral blood indicate MAHA also renal failure is indicated

LDH is indicative of tissue breakdown

Hb in circulation so is intravascular

HUS

98
Q

Mechanical damage induced hemolytic anemia

99
Q

Malaria can also cause?

A

Hemolytic anemia

100
Q

Prognosis of hemolytic anemia

101
Q

Summary of extrinsic hemolysis

102
Q

Coomb’s positive vs negative

103
Q

summary of WAIHA

104
Q

Summary of CAIHA

105
Q

Summary of PCH

106
Q

Summary of MAHA

Hematology (26 decks)

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