Myelofibrosis Flashcards
What is myelofibrosis?
Neoplastic proliferation of mature myeloid cells - in particular megakaryocytes
What are risk factors for myelofibrosis?
Age >65
Radiation
What is the pathophysiology of myelofibrosis?
JAK2 mutation -> proliferation of megakaryocytes
Release platelet-derived growth factor
- marrow fibrosis
- extramedullary haematopoiesis
What investigations are done in suspected myelofibrosis?
FBC
Blood film
Bone marrow aspirate
Bone marrow biopsy
What does FBC show in myelofibrosis?
Anaemia
What does the blood film show in myelofibrosis?
Leucoerthroblastic smear
- tear drop RBCs
- nucleated RBCs
What does a bone marrow aspirate show in myelofibrosis?
Dry tap
What does a bone marrow biopsy show in myelofibrosis?
Marrow fibrosis
How is myelofibrosis managed?
Chemotherapy
What are features of myelofibrosis?
Anaemia
Massive splenomegaly
B symptoms