Medicine

Question 1

What is the mutation found in polycythaemia rubra vera?

Answer 1

JAK2

Question 2

What is myelofibrosis?

Answer 2

Myelofibrosis is where the proliferation of the cell line leads to fibrosis of the bone marrow leading to anaemia and leukopaenia

Question 3

What are the causes of myelofibrosis?

Answer 3

Primary myelofibrosis, secondary to another myeloproliferative disorder such as polycythaemia vera or essential thrombocytosis

Question 4

What are the blood film findings in myelofibrosis?

Answer 4

Teardrop-shaped RBCs, varying sizes of red blood cells (poikilocytosis) and immature red and white cells (blasts).

Question 5

What are the complications of myelofibrosis?

Answer 5

Gout, bleeding, thrombosis, transformation to AML

Question 6

How is myelofibrosis treated?

Answer 6

Watch and wait, stem cell transplantation, chemotherapy.

If secondary to ET or PV - aspirin also

Question 7

What are the clinical features of a myeloproliferative disorder?

Answer 7

Fatigue, weight loss, night sweats, fever, anaemia (unless polycythaemia), splenomegaly, portal hypertension, petechiae, plethoric complexion