Leukemia and other myeloid neoplasms

Question 1

What are the criteria for high-risk ET

Answer 1

1 or more of 3 major risk factors:

1. age > 60 years
2. history of thrombosis or major bleeding
3. platelet count > 1500 x 10^9/L

Question 2

What are the criteria for low-risk ET

Answer 2

1. age < 60 years
2. no history of thrombosis or major bleeding
3. platelet count< 1500 x 10^9/L

none of the 3 major risk factors cardiovascular risk factors: diabetes, dyslipidemia, active tobacco use, and hypertension.

Question 3

What is the treatment of patients with low risk ET?

Answer 3

Observation

Question 4

What is the treatment of patients with low risk ET + cardiovascular risk factors?

Answer 4

Low dose aspirin

Question 5

What is the treatment of patients with high risk ET?

Answer 5

Aspirin and hydroxyurea

Question 6

What is the most likely dx in a young male patient with pancytopenia and splenomegaly?

Answer 6

Hairy cell leukemia

Question 7

What is the characteristic immunophenotype for hairy cell leukemia?

Answer 7

Hairy cells express bright, monotypic surface immunoglobulin, CD19, bright CD20, CD22, and CD11c, in addition to CD25, CD103, CD123, and FMC7

Question 8

What is the most common mutation found in classic hairy cell leukemia?

Answer 8

BRAF V600E mutation is uniformly positive in the classic HCL

Question 9

What are the indications for treatment of hairy cell leukemia?

Answer 9

Treatment is generally initiated when there are significant cytopenias, symptomatic splenomegaly, or symptoms (fever, night sweats, or recurrent infections)

Question 10

What is the treatment of hairy cell leukemia?

Answer 10

Treatment is generally initiated when there are significant cytopenias, symptomatic splenomegaly, or symptoms (fever, night sweats, or recurrent infections)