Primary Myelofibrosis

Question 1

what is myelofibrosis

Answer 1

It is characterised by abnormal production of red blood cells, white blood cells, and platelets, in association with marrow fibrosis (scarring) and extramedullary haematopoiesis and splenomegaly

Question 2

aetiology of myelofibrosis

Answer 2

It results in increased numbers of abnormal megakaryocytes with stromal proliferation secondary to growth factors released by megakaryocytes

Question 3

risk factors for myelofibrosis

Answer 3

radiation exposure, industrial solvents exposure

age above 65

Question 4

how does myelofibrosis present

Answer 4

Constitutional symptoms: weight loss, fever, night sweats, cachexia and fatigue

Splenomegaly: abdominal discomfort, early satiety

Marrow failure: anaemia, recurrent infection, and abnormal bleeding/bruising.

Hepatomegaly: seen in 50% of patients.

Question 5

what investigations will be done to diagnose myelofibrosis

Answer 5

On blood film, you would expect to see poikilocytes (tear-shaped red blood cells).

Because of the marrow fibrosis, it is difficult to aspirate the bone marrow, resulting in a ‘dry tap’

bone marrow biopsy will show fibrosis

Question 6

management of myelofibrosis

Answer 6

Treatment involves stem cell transplantation or

immunomodulatory drugs such as Thalidomide