5. Hemolytic anemia

Question 1

diagnostic evaluation of hemolytic anemia

Answer 1

normocytic, normochromic anemia
RI> 2%, ↑ LDH, ↓ Haptoglobin, ↑ indirect bilirubin
intravascular hemolysis: schistocytes on peripheral smear
extravascular: splenomegaly
autoimmune hemolysis: coomb’s test

Question 2

intrinsic RBC defect (hereditary)

Answer 2

enzyme deficiency: G6PD
hemoglobinopathies: sickle cell disease, thalassemia
membrane abnormalities: hereditary spherocytosis, PNH

Question 3

extrinsic RBC defect (acquired)

Answer 3

immune: AIHA, drug induced anemia
traumatic: micro/macroangiopathic HA
infections: malaria, babesiosis

Question 4

what precipitates hemolysis in G6PD deficiency?

Answer 4

drugs (sulfonamides, dapsone)
infection
DKA
fava beans

Question 5

diagnosis of sickle cell disease

Answer 5

sickle shaped RBC’s and Howell-holly bodies on smear

Hgb electrophoresis

Question 6

management of sickle cell disease

Answer 6

• hydroxyurea

- allogenic HSCT (young patient with severe disease)

Question 7

clinical manifestations of hereditary spherocytosis

Answer 7

anemia, jaundice, splenomegaly, pigmented gallstones

Question 8

diagnosis of hereditary spherocytosis

Answer 8

• spherocytes on blood smear
• ↓ mean fluorescence of RBC’s in EMA binding test
• ↑ fragility in osmotic fragility test

Question 9

management of hereditary spherocytosis

Answer 9

folate supplementation

splenectomy in severe disease

Question 10

triad seen in paroxysmal nocturnal hemoglobinuria (PNH)?

Answer 10

1) intravascular hemolytic anemia (coomb’s negative)
2) hypercoagulability (venous > arterial, intrabdominal, cerebral)
3) pancytopenia

*hemoglobinuria, jaundice, IDA

Question 11

late consequences of PNH

Answer 11

Aplastic anemia
MDS and evolution to AML
Budd-Chiari syndrome

Question 12

management of PNH

Answer 12

• allogenic HSCT (if hypoplasia or severe thrombosis
• Eculizumab (Ab inactivates terminal complement C5s)–> ↓ Hemolysis
• glucocorticoids (initial presentation)
• splenectomy (if progresses to extravascular hemolysis)
• prophylaxis anticoagulation

Question 13

difference between warm and cold AIHA

Answer 13

Warm: IgG Ab’s opsonize RBC’s at body temp–> removal by spleen
cold: IgM Ab’s bind to RBCs at temp<37–> complement fixation–> intravascular hemolysis

Question 14

in what diseases can warm AIHA be seen?

Answer 14

autoimmune- SLE
drugs
CLL, NHL

Question 15

in what diseases can cold AIHA be seen?

Answer 15

mycoplasma pneumonia

infectious mononucleosis

Question 16

treatment for warm and cold AIHA

Answer 16

Warm: corticosteroids, splenectomy, IVIG, rituximab
cold: avoid cold, maybe rituximab

Question 17

what is the result of coombs test in drug induced hemolytic anemia?

Answer 17

usually negative

Question 18

what drugs cause drug-induced hemolytic anemia?

Answer 18

Ab: cephalosporin, sulfa drugs

cardio: methyldopa, procainamide
other: TCA’s, NSAID’s

Question 19

causes of splenomegaly

Answer 19

hemolytic anemia, SCD
infections (CMV, malaria) 
autoimmune (SLE)
serum sickness, 
cirrhosis, CHF
lysosomal/glycogen storage diseases
acute leukemia, lymphoma