BMF II

Question 1

the most common form of hereditary nonspherocytic hemolytic anemia

Answer 1

PYRUVATE KINASE DEFICIENCY

Question 2

is due to mutation in the PKLR gene

Answer 2

is due to mutation in the PKLR gene

Question 3

metabolic consequence: depletion of ATP and an increase in 2,3 - BPG

Answer 3

is due to mutation in the PKLR gene

Question 4

are conditions in which RBC survival is shortened due to an antibody-mediated mechanism. The antibody may be an autoantibody, an alloantibody, or an antibody directed against a drug taken by the patient

Answer 4

IMMUNE HEMOLYTIC ANEMIAS

Question 5

is the most commonly encountered autoimmune hemolytic anemia

Answer 5

Warm autoimmune hemolytic anemia

Question 6

the autoantibodies react optimally at 37°C

Answer 6

Warm autoimmune hemolytic anemia

Question 7

onset is usually insidious, with symptoms of anemia

Answer 7

Warm autoimmune hemolytic anemia

Question 8

hemolysis is predominantly extravascular

Answer 8

Warm autoimmune hemolytic anemia

Question 9

Typical findings on the PBS of warm autoimmune hemolytic anemia

Answer 9

polychromasia and spherocytes

Question 10

IgM class that react optimally at 4°C and are commonly found in healthy individuals

Answer 10

Cold agglutinin disease

Question 11

pathologic cold agglutinins can react at

Answer 11

Body temp

Question 12

hemolysis is predominantly extravascular by hepatic macrophages

Answer 12

Cold agglutinin disease

Question 13

symptoms include acrocyanosis (a bluish discoloration of the extremities)

Answer 13

Cold agglutinin disease

Question 14

is due to a biphasic IgG autoantibody with an anti-P specificity

Answer 14

Paroxysmal cold hemoglobinuria

Question 15

at cold temperatures, the antibody binds to the P antigen on RBCs and partially activates complement

Answer 15

Paroxysmal cold hemoglobinuria

Question 16

full complement activation and hemolysis occur only upon warming to 37°C

Answer 16

Paroxysmal cold hemoglobinuria

Question 17

is suspected when there is a sudden decrease in hemoglobin after administration of a drug, clinical and biochemical evidence of extravascular or intravascular hemolysis, and a positive DAT results.

Answer 17

Drug-induced immune hemolytic anemia

Question 18

unifying theory: proposes that a drug interacts with the RBC membrane and generates multiple immunogenic epitopes that can elicit an immune response to the drug alone, to the drug-RBC membrane protein complex, or to an RBC membrane protein alone

Answer 18

Drug-induced immune hemolytic anemia