Hemolytic Anemia

Question 1

What are the hemoglobinopathies?

Answer 1

Thalassemia
Sickle cell disease
Congenital dyserythropoetic anemia
Hemolytic disease of the newborn

Question 2

What is the most common enzymopathy?

Answer 2

Glucose 6 phosphate dehydrogenase deficiency

Can be flared up by drugs and/or stress

Question 3

What are some drugs that can flare up G6PD deficiency?

Answer 3

Antimalarials : primaquine (mostly) and chloroquine (often)
Sulfamethoxazole
Nitrofurantoin

Question 4

What are the proteins affected in an abnormal RBC cytoskeleton?

Answer 4

Ankyrin
Spectrin
Band 3

Question 5

What are the cytoskeletal causes for hemolysis?

Answer 5

Hereditary spherocytosis- diagnosed with osmotic fragility test
Hereditary elliptocytosis
Stomacytosis
Xerocytosis

Question 6

What are the causes of acquired hemolytic anemia?

Answer 6

Infection

Mechanical destruction

Question 7

What are the most common infections causing acquired hemolytic anemia?

Answer 7

Malaria
Babesiosis
Clostridium perfringens (lecithinase)
Shiga toxin (E coli O157:H7->HUS)

Question 8

What are the mechanical destruction leading to acquired hemolytic anemia?

Answer 8

Prosthetic heart valves- most common

Microangiopathic hemolytic anemia (MAHA)/Thrombotic thrombocytopenic purpura (TTP)- ADAMTS-13 defect

Question 9

What are the antibodies associated with acquired immune hemolytic anemia?

Answer 9

Viral infection: EBV, CMV

Other disease: SLE, CLL

Question 10

What is the presentation of paroxysmal nocturnal hemoglobinuria (PNH)?

Answer 10

Hemolysis
Pancytopenia
Venous thrombosis

Question 11

What are the common features of hemolytic disorder?

Answer 11

Reticulocytes- increased
Unconjugated bilirubin- increased
LDH- increased
Haptoglobin- reduced to absent