Myeloproliferative Disorders (3)

Question 1

What occurs here?

What can it progress into?

What are its 3 main types?

Answer 1

➊ Uncontrolled proliferation of 1 stem cell type

➋ AML

➌ • Primary Myelofibrosis - Proliferation of haematopoietic stem cells, and characterised by bone marrow fibrosis. Blood film shows poikilocytes (tear-shaped RBCs).
• Polycythaemia Vera - Proliferation of erythroid cell line, presenting with hyperviscocity syndrome (chest pain, myalgia, visual disturbances), and associated with JAK-2 mutation
• Essential Thrombocytosis - Proliferation of megakaryocytic cell line (platelet precursor)

N.B. Myelofibrosis will cause a ‘dry tap’