Primary Myelofibrosis Flashcards
1
Q
What is the mutation in primary myelofibrosis?
A
Jak2 mutation on the short arm of chromosome 9.
2
Q
WHat happens?
A
deposition of type 3 collage due to abnormla megakaryocytes secreting cytokines. leads to extramedullary tumors
3
Q
How do patients present?
A
affects people over the age of 50.
Present with splenomegaly, portal hypertension, bone pain, thrombosis.
4
Q
Peripheral blood?
A
weird looking megs, abnormal plateletts, tear drop and nucleated rbc.
5
Q
What does the bone marrow look like?
A
Full of type 3 collagen
6
Q
Diagnose with?
A
reticulin stain for fibers.
7
Q
Treat?
A
supportive care.
