Hematology Week 1: Extrinsic Hemolytic Anemias

Question 1

Extrinsic Hemolytic Anemias Definition

Answer 1

Destruction of RBCs from extracellular etiology

Question 2

Major types of Extrinsic Hemolysis

Answer 2

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

Question 3

WarmAIHA DAT Test

Answer 3

IgG

Question 4

ColdAIHA DAT Test

Answer 4

IgM, C3

Question 5

PCH DAT Test

Answer 5

Biphasic polyclonal IgG

Question 6

WAIHA Etiology

3 listed

Answer 6

• 50% Idiopathic
• LPD
• Autoimmune Diseases

Question 7

WAIHA T of Ab binding

Answer 7

37*C

Question 8

WAIHA T of Hemolysis

Answer 8

37*C

Question 9

WAIHA Site of Hemolysis

Answer 9

• Extravascular
• Spleen macrophages

Question 10

WAIHA Treatment

Answer 10

Glucocorticoids 1*

Splenectomy 2*

Question 11

CAIHA Etiology

Answer 11

• LPD (CLL)
• Infections (Mycoplasma pneumonia, EBV, HIV)

Question 12

CAIHA T of Ab binding

Answer 12

1-5*C

Question 13

CAIHA T of Hemolysis

Answer 13

37*C

Question 14

CAIHA Site of hemolysis

Answer 14

• Extravascular
• Liver

Question 15

CAIHA Treatment

3 listed

Answer 15

• Avoid Cold
• Supportive
• Treat underlying disease

Question 16

PCH Etiology

Answer 16

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

Question 17

PCH T of Ab binding

Answer 17

Cold

Question 18

PCH T of Hemolysis

Answer 18

37*C

Question 19

PCH Site of Hemolysis

Answer 19

Intravascular Sudden and Massive

Question 20

PCH Treatment

Answer 20

Supportive care

Question 21

MAHA AKA

Answer 21

Microangiopathic Hemolytic Anemia

Question 22

Types of MAHA

3 listed

Answer 22

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

Question 23

IHA AKA

Answer 23

Immune-mediated Hemolytic Anemia

Question 24

Immune-mediated Hemolytic Anemia

Answer 24

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

Question 25

DAT AKA

Answer 25

Direct antiglobulin test or Coombs test

Question 26

Blood Findings of IHA 4 listed

Answer 26

Question 27

Spherocytes seen in? 2 listed

Answer 27

* IHA * Hereditary spherocytosis

Question 28

WAIHA Overview

Answer 28

Question 29

Etiology of WAIHA

Answer 29

Question 30

most common tupe of IHA

Answer 30

WAIHA

Question 31

1/3 of SLE patients have?

Answer 31

WAIHA

Question 32

Pathogenesis of WAIHA

Answer 32

Question 33

WAIHA Extravascular or Intravascular?

Answer 33

Extravascular

Question 34

PB Smear in WAIHA

Answer 34

* multiple spherocytes * Spherocytes do not have central pallor

Question 35

WAIHA Clinical Presentations 4 listed

Answer 35

Question 36

Treatment of WAIHA 6 listed

Answer 36

Question 37

Definition of CAIHA

Answer 37

Question 38

CAIHA Intravascular or Extravascular

Answer 38

Extravascular hemolysis in the liver and can occur intravascularly!

Question 39

Etiology CAIHA

Answer 39

Question 40

Common causes of CAIHA

Answer 40

* Chronic Lymphocytic Leukemia (CLL) * Mycoplasma Pneumonia * EBV and HIV * SLE * Idiopathy * Cancer

Question 41

Clinical Manifestations of Cold Agglutination Disease CAIHA

Answer 41

Question 42

CAIHA AKA

Answer 42

Cold Agglutination Disease

Question 43

Unique Laboratory findings in CAIHA 4 listed

Answer 43

Question 44

Special test for CAIHA

Answer 44

Cold agglutinin titer will be elevated in CAIHA

Question 45

PB smear in CAIHA

Answer 45

clumps of RBCs from IgM linking

Question 46

Treatment of CAIHA

Answer 46

Question 47

Most common cause of CAIHA

Answer 47

CLL

Question 48

PCH AKA

Answer 48

Paroxysmal Cold Hemoglobinuria

Question 49

PCH Etiology

Answer 49

* 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

Question 50

PCH Pathogenesis 4 listed

Answer 50

* 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

Question 51

Donath-Landsteiner Antibody

Answer 51

PCH

Question 52

Clinical presentation of PCH

Answer 52

Question 53

Blood fings in PCH

Answer 53

neutrophils phagocytosing RBCs is characteristic

Question 54

Unique Lab test for PCH

Answer 54

Donath Landsteiner Test

Question 55

Unique Lab findings in PCH

Answer 55

Question 56

Drug-induced hemolytic anemia

Answer 56

Question 57

Treatment of PCH

Answer 57

Question 58

Most common drug causing drug-induced hemolytic anemia

Answer 58

Cephalosporin

Question 59

Drug-induced hemolytic anemia subsets

Answer 59

Question 60

Drug-induced hemolytic anemia subsets most common?

Answer 60

penicillins/cephalosporin

Question 61

Specific test for Drug-induced hemolytic anemia

Answer 61

None

Question 62

Lab findings of Drug-induced Hemolytic Anemia

Answer 62

Question 63

Comparison of WAIHA, CAIHA PCH

Answer 63

cold is extra and intravascular!!!!!!

Question 64

Question 1

Answer 64

E Microspherocytes

Question 65

when do you see macroovalocytes?

Answer 65

in foltae and B12 difficiency anemia

Question 66

When do you see hypochromic microcytic cells?

Answer 66

Iron Deficiency Anemia

Question 67

When do you see polychromatic cells?

Answer 67

Any time a patient has anemia except for Iron Deficiency Anemia

Question 68

When do you see Schistocytes?

Answer 68

intravascular hemolysis

Question 69

When do you see microspherocytes?

Answer 69

Immune-mediated hemolytic Anemias

Question 70

Question 2

Answer 70

B Idiopathic

Question 71

Question 3

Answer 71

D Extravascular spleen will see splenomegaly

Question 72

MAHA AKA

Answer 72

Microangiopathic hemolytic anemia

Question 73

MAHA characteristic blood finding

Answer 73

schistocytes

Question 74

Major Types of MAHA 6 listed

Answer 74

most common are? Disseminated intravascular coagulation (DIC) Hemolytic Uremic Syndrome (HUS) Thrombotic Thrombocytopenic Purpura (TTP)

Question 75

2 common pathways of intravascular hemolysis

Answer 75

Question 76

Lab findings in MAHA 8 listed

Answer 76

Question 77

DIC AKA

Answer 77

Disseminated Intravascular Coagulopathy

Question 78

DIC Etiology

Answer 78

DIC IS A MEDICAL EMERGENCY

Question 79

DIC Pathogenesis

Answer 79

Question 80

Lab findings in DIC 4 listed

Answer 80

Question 81

D-dimer in DIC

Answer 81

Significantly increased

Question 82

Fibrinogen in DIC

Answer 82

Decreased fibrinogen level

Question 83

TTP AKA

Answer 83

Thrombotic thrombocytopenic Pupura (TTP)

Question 84

TTP Definition

Answer 84

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

Question 85

Hereditary TTP

Answer 85

Mutations of ADAMTS13 genes Markedly decreased ADAMTS13 level

Question 86

Acquired TTP

Answer 86

Autoimmune phenomenon associated with malignancy, HSCT, pregnancy, medications or infection such as HIV Markedly decreased ADAMTS13 level Anti ADAMTS13 antibody

Question 87

Commonality of Hereditary TTP and Acquired TTP

Answer 87

Markedly decreased levels of ADAMTS13 levels

Question 88

Function of Von Willebrand Factor

Answer 88

ADAMTS13 cuts vWF when deficient causes coagulation

Question 89

TTP Pathogenesis

Answer 89

extra von Willebrand factor (vWF)

Question 90

Mortality in TTP

Answer 90

90% mortality if untreated

Question 91

Plasmic score

Answer 91

indivative of TTP

Question 92

HUS AKA

Answer 92

Hemolytic Uremic Syndrome

Question 93

Typical HUS

Answer 93

Shiga-like toxin-associated HUS (Stx-HUS) infections with E. coli in 75% of cases Enterococcus or Shigella in some cases

Question 94

Atypical HUS

Answer 94

* 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

Question 95

Comparison of DIC, TTP and HUS

Answer 95

Question 96

Clinical Features of HUS 5 listed

Answer 96

Question 97

Question 5

Answer 97

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

Question 98

Mechanical damage induced hemolytic anemia

Answer 98

Question 99

Malaria can also cause?

Answer 99

Hemolytic anemia

Question 100

Prognosis of hemolytic anemia

Answer 100

Question 101

Summary of extrinsic hemolysis

Answer 101

Question 102

Coomb's positive vs negative

Answer 102

Question 103

summary of WAIHA

Answer 103

Question 104

Summary of CAIHA

Answer 104

Question 105

Summary of PCH

Answer 105

Question 106

Summary of MAHA

Answer 106