Aplastic anemia

Question 1

Common associated condition with aplastic anemia

Answer 1

Thymoma

Question 2

Triggers for aplastic anemia

Answer 2

• drugs - benzenes,
• hepatitis
• EBV, parvo, HIV

Question 3

Bone marrow findings in aplastic anemia

Answer 3

• pancytopenia with a profoundly hypocellular bone marrow
• bone marrow replaced by fat cells and marrow stroma
  **see photo online
• no abnormal infiltrate
• no marrow fibrosis.

Question 4

Most common mutations in aplastic anemia

Answer 4

BCOR (1)
TET2 (2)

Question 5

aplastic anemia treatment

Answer 5

• IF <40, matched donor transplant
• IF older, immunosuppressive t cell targeted therapy:
  Triple IST (Equine ATG + cyclosporine A + eltrombopag) (RACE)
  *cyclosporine slowly tapered off if responding

Question 6

ATG SE’s to know

Answer 6

• infusion reactions
• anaphylaxis
• serum sickness

Question 7

Serum sickness prophylaxis and treatment

Answer 7

steroids - prednisone

Question 8

Goal cyclosporine

Answer 8

200-400

Question 9

screening required for aplastic anemia patients

Answer 9

• yearly screening for PNH clone

Question 10

Potential sequela of eltrombopag use in aplastic anemia

Answer 10

Low but significant risk of clonal evolution

Question 11

second line for aplastic anemia

Answer 11

• allo-HSCT
• alternative immunosuppressants - rabbit ATG, alemtuzumab

Question 12

aplastic anemia sequela

Answer 12

• clonal hematopoesis

Question 13

Management of PNH clone in aplastic anemia

Answer 13

• most are subclinical and just observed
  IF large or symptomatic, treat with complement inhibitors