MPD

Question 1

What allows differentiation between acute leukaemia and myeloproliferative disorders?

Answer 1

Cells in MPD retain the ability to mature and differentiate

Question 2

What predisposes individuals with MPD to gout?

Answer 2

Increased cell turnover resulting in and increase of purine breakdown

Question 3

What is the Philadelphia chromosome and what affect does it have on patient outcome?

Answer 3

Translocation of genes 9 and 22, forming the new gene BCR-ABL1; gives a better prognosis.

Question 4

PRV should be distinguished from what 2 conditions? Give an example for each

Answer 4

• Secondary polycythaemia; chronic hypoxia.

- Pseudopolycythaemia; dehydration

Question 5

Genetic testing looking for what particular gene has made EPO levels and bone marrow biopsy no longer routine tests?

Answer 5

JAK2

Question 6

What investigations/aspects of the Hx can help look for secondary/pseduo cases of PRV?

Answer 6

• CXR
• O2 sats
• ABGs
• DHx

Question 7

In low risk individuals with PRV, what is the main method of management?

Answer 7

Venesection to maintain haematocrit below 0.45 to reduce risk of thrombosis

Question 8

What causes the significant organomegaly seen in myelofibrosis?

Answer 8

Intense marrow fibrosis, causing extra medullary haematopoiesis in the liver and spleen

Question 9

Blood film of patient with suspected myelofibrosis would show which sign?

Answer 9

Tear-drop shaped RBC