Myeloproliferative/Myelodysplastic Disorder

Question 1

What are the three myeloproliferative disorders and what cells do they affect primarily?

Answer 1

1. Primary myelofibrosis - haematopoietic stem cell
2. Polycythaemia Vera - erythroid cells
3. Essential thrombocytopenia - megakaryocyte

Question 2

What genetic mutation are myeloproliferative disorders associated with?

Answer 2

JAK2 (target of JAK2 inhibitors like ruxolitinib)

Question 3

What is myelofibrosis?

Answer 3

Cell proliferation leads to bone marrow fibrosis, causing anaemia and leukopenia.

Question 4

Where does haematopoiesis occur in myelofibrosis?

Answer 4

Liver and spleen (both become enlarged)

Question 5

What is the management of myeloproliferative disorders?

Answer 5

1. Primary myelofibrosis - allogenic stem cell transplant, chemotherapy, supportive management
2. Polycythaemia Vera - venesection, aspirin, chemotherapy
3. Essential thrombocytopenia - aspirin, chemotherapy

Question 6

What is myelodysplastic syndrome?

Answer 6

Myeloid cells don’t mature properly which causes anaemia, neutropenia, thrombocytopenia. Risk of transformation into AML.

Question 7

What are the primary causes of secondary bone metastases?

Answer 7

BLT Plus Ketchup
B - breast
L - lung
T - thyroid
P - prostate - osteosclerotic lesions, no high calcium
K - kidney