Hemolytic anemia

Question 1

3 general levels of diagnosing hemolytic anemia

Answer 1

Clinical suspicion
Screening lab tests
Precise identification of cause

Question 2

5% or more increase in reticulocytes suggests what

Answer 2

Hemolysis of RBCs

Question 3

Presence of blister cells and Heinz bodies suggests what

Answer 3

G6PD deficiency

Question 4

Presence of Howell-Jolly bodies is seen in what two anemias

Answer 4

Sickle cell
Severe hemolytic anemia

Question 5

Increased indirect bilirubin and LDH is seen in what category of anemias

Answer 5

Hemolytic anemias

Question 6

Anemia that primarily involves phagocytosis by macrophages in the RE system

Answer 6

Extravascular hemolytic anemia

Question 7

3 causes of intravascular hemolytic anemia

Answer 7

Mechanical injury
Complement fixation
Exogenous toxin

Question 8

Inherited mutations affecting membrane proteins in RBCs, particularly spectrin and ankyrin

Answer 8

Hereditary spherocytosis

Question 9

Inheritance of hereditary spherocytosis

Answer 9

Autosomal dominant

Question 11

Possible complication in children with hereditary spherocytosis

Answer 11

Aplastic crisis after viral infection

Question 12

Inheritance of G6PD deficiency

Answer 12

X-linked recessive

Question 13

Reduced ability of RBC to protect against oxidative injury leading to hemolysis

Answer 13

G6PD deficiency

Question 14

Heinz bodies and bite cells are seen in what anemia

Answer 14

G6PD deficiency

Question 15

Inheritance of pyruvate kinase deficiency

Answer 15

Autosomal recessive

Question 16

Mechanism of reduced clinical severity of pyruvate kinase deficiency

Answer 16

2,3-BPG is increased causing a right-shift of the O2 dissociation curve, increasing the release of O2 in the tissue

Question 17

Echinocytes on smear is associated with what hemolytic anemia

Answer 17

Pyruvate kinase deficiency

Question 18

Confirmatory test for pyruvate kinase deficiency

Answer 18

RBC enzyme assay

Question 19

Sickle cell anemia mutation

Answer 19

Point mutation in 6th beta-globin gene from glutamate to valine results in HbS

Question 20

93% A, 2% F and 5% A2 on Hb electrophoresis

Answer 20

Beta-thalassemia minor

Question 21

90% F and 10% A2 on Hb electrophoresis

Answer 21

Beta-thalassemia major

Question 22

Number of affected alpha-globin genes in alpha-thalassemia minor

Answer 22

2

Question 23

Number of affected alpha-globin genes in HbH disease

Answer 23

3

Question 24

Due to acquired mutations in PIG-A, an intermediary of GPI, on the X-chromosome

Answer 24

Paroxysmal nocturnal hemoglobinuria

Question 25

Function of GPI linked proteins

Answer 25

Prevent auto-activation of the alternative complement pathway

Question 26

Proteins normally linked to GPI

Answer 26

CD55
CD59

Question 27

Triad of clinical features in PNH

Answer 27

Intravascular hemolysis
Thrombosis
Bone marrow hypoplasia/aplasia

Question 28

Cause of intravascular hemolysis in PNH

Answer 28

C5b-C9 membrane attack complex

Question 29

Leading cause of death in PNH

Answer 29

Thrombosis

Question 30

Possible progressions of PNH

Answer 30

Aplastic anemia
AML

Question 31

DAT positive with anti-IgG/C3d with spherocytes in smear

Answer 31

Warm autoimmune hemolytic anemia

Question 32

Treatment option for both types of autoimmune hemolytic anemias

Answer 32

Rituximab

Question 33

Treatment for paroxysmal cold hemaglobinuria

Answer 33

Plasma exchange therapy to remove bithermal antibody