Myeloproliferative Disorders - General

Question 1

Aetiology of Myeloproliferative Disorders.

Answer 1

Uncontrolled proliferation of a single type of stem cell - a type of bone marrow cancer.

Question 2

Genetics of Myeloproliferative Disorders.

Answer 2

Mutations in JAK2, MPL and CALR genes.

Question 3

Aetiology of Myelofibrosis.

Answer 3

A result of any of the 3 myeloproliferative disorders.

Question 4

What is Myelofibrosis?

Answer 4

Proliferation of the cell line leads to fibrosis (replacement by scar tissue) of the bone marrow, in response to cytokines e.g. FGF released from proliferating cells.

Question 5

Clinical Consequence of Myelofibrosis.

Answer 5

Extramedullary Haematopoeisis - Massive Hepatomegaly or Splenomegaly (Portal Hypertension, Spinal Cord Compression).

Question 6

Blood Film of Myelofibrosis.

Answer 6

Tear-Drop Shaped RBCs = Poikilocytosis and Immature Red/White Blood Cells (Blasts).

Question 7

Diagnosis of Myeloproliferative Disorders.

Answer 7

Bone Marrow Biopsy - Usually Bone Marrow Aspiration is Dry.