MPN - Myeloproliferative Neoplasms

Question 1

What are the 4 major conditions in MPN?

Answer 1

1. CML - Chronic Myeloid Leukaemia
2. Polycythaemia Vera
3. Essential thrombocythaemia
4. Primary Myelofibrosis

Question 2

What is the effect of excessive alcohol consumption on platelet count, and haemoglobin (Hb)?

Answer 2

Excessive alcohol consumption can decrease platelet count and increase Hb.

Question 3

What are the causes of secondary/reactive thrombocythaemia?

Answer 3

1. Infection: most common of which are pneumonia, meningitis, UTI, etc.
2. Inflammation: Inflammatory conditions incl IBD or Rheumatoid arthritis
3. Trauma: burns
4. Post-splenectomy or hyposplenism

Question 4

What investigative factors are likely to be raised in secondary thrombocythaemia?

Answer 4

ESR, CRP, Fibrinogen levels, Factor VIII pro-coagulant activity and Von Willebrand antigen level.

Question 5

What are the hallmark features of Essential thrombocythaemia?

Answer 5

1. Sustained rise in thrombocytes/platelets >600 x 10^9/L.
2. Megakaryocyte hyperplasia
3. Splenomegaly
4. an increased tendency to both thrombosis and bleeding

Question 6

Which condition is positive for the Philadelphia chromosome?

Answer 6

Chronic Myeloid Leukaemia

Question 7

What is the Philadelphia chromosome?

Answer 7

A translocation mutation between chromosomes 9 and 22, resulting in BCR-ABL1 gene.

Question 8

Antiphospholipid antibodies increase/decrease risk of thrombosis?

Answer 8

Antiphospholipid antibodies increases risk of thrombosis.

Question 9

Is the EPO level in PV high or low?

How about in secondary polycythaemia?

Answer 9

EPO level in PV is low.

EPO is high in secodnary polycythaemia.