Myeloproliferative Disorders

Question 1

What are Myeloproliferative Disorders

Answer 1

Uncontrolled proliferation of a single type of stem cell

Question 2

The three myeloproliferative disorders

Answer 2

Primary myelofibrosis

Polycythaemia vera

Essential thrombocythaemia

Question 3

What is Primary myelofibrosis?

Answer 3

Proliferation of the hematopoietic stem cells

Question 4

Polycythaemia vera

Answer 4

Proliferation of the erythroid cell line

Question 5

Essential thrombocythaemia

Answer 5

Proliferation of the megakaryocytic cell line

Question 6

Myeloproliferative Disorders associated with what mutations

Answer 6

JAK2
MPL
CALR

Question 7

What is myelofibrosis?

Answer 7

Result of primary myelofibrosis, polycythaemia vera or essential thrombocythaemia

Proliferation of the cell line leads to fibrosis of the bone marrow in response to cytokines

Bone marrow is replaced with scar tissue the production of blood cells starts to happen in other areas (liver/spleen)

Question 8

Signs of polycythaemia vera on examination

Answer 8

Conjunctival plethora (excessive redness to the conjunctiva in the eyes)

A “ruddy” complexion

Splenomegaly

Question 9

Blood findings in Polycythaemia Vera

Answer 9

Raised haemoglobin (more than 185g/l in men or 165g/l in women)

Question 10

Blood findings in Primary Thrombocythaemia

Answer 10

Raised platelet count (more than 600 x 109/l)

Question 11

Blood findings in Myelofibrosis can give variable findings:

Answer 11

Anaemia

Leukocytosis or leukopenia

Thrombocytosis or thrombocytopenia

blood film in myelofibrosis can show teardrop-shaped RBCs and poikilocytosis

Question 12

Diagnosis of Myelofibrosis

Answer 12

bone marrow biopsy