Primary Myelofibrosis

Question 1

What is the overall problem with myelofibrosis?

Answer 1

Too many dysplastic megakaryocytes stimulating collagen production leading to fibrosis in the bone marrow.

Question 2

the fibrosis leads to what, which shows what on physical exam, what does peripheral smear show, what does bone marrow biopsy show, and what is the mutation?

Answer 2

Extramedullary blood production
Big liver and spleen
Leukoerythroblastic with immature WBCS, uncleared RBCs, and tear drop RBCs
Dry tap but collagen and dysplastic megekaryocytes
Jak2

Question 3

What chromosomal abnormalities are associated with fibrosis?

Answer 3

9p, 20q, 13q and trisomy 8 or 9.

Question 4

For PMF treatment, what is used to treat the anemia? Control splenomegaly? Control significant hyperuricemia? Help with fibrosis? Only thing that is curative?

Answer 4

Danazol, but often transfusions are required
Hydroxyurea or jak2 inhibitor 
Allopurinol
IFN alpha 
Bone marrow transplant

Question 5

What is the hallmark of PCV and what two things commonly accompany it?

Answer 5

Erythrocytosis

Leukocytosis and thrombocytosis

Question 6

What is the mutation of PCV and two things to treat?

Answer 6

Jak2

Phlebotomy and hydroxyurea