Myelofibrosis

Question 1

What is myelofibrosis

Answer 1

Myelofibrosis (MF) is a chronic myeloproliferative neoplasm characterized by the gradual replacement of normal bone marrow tissue with fibrous tissue, caused by KAK2 mutation

Question 2

How

Question 3

How does Myelofibrosis appear on blood film?

Answer 3

tear-shaped poikilocytes and a leucoerythroblastic blood film
Dry tap on bone marrow aspirate

Question 4

How does it present clinically?

Answer 4

Constitutional symptoms – weight loss, fever, night sweats
Marrow failure – anaemia, recurrent infection, and abnormal bleeding/bruising
Bone pain
Haemorrhage and thrombosis (less common in myelofibrosis than in other myeloproliferative disorders)
Signs include:
Splenomegaly (can be massive and painful) – abdominal discomfort, early satiety
Hepatomegaly (seen in 50% of patients)

Question 5

How is myelofibrosis managed?

Answer 5

Allogenic stem cell transplantation with immunomoduatory therapy of thalidmoide

JAK2 inhibtiors like ruxolitinib

Cytotoxic agents

Question 7

What is the blood film finding for myelofibrosis?

Answer 7

Poikilocytes which are tear drop RBCs