Myeloproliferative disorders

Question 1

What are myeloproliferative disorders?

Answer 1

• Uncontrolled proliferation of a single stem cell

Question 2

What are the three myeloproliferative disorders?

Answer 2

• Primary Myelofibrosis (Haematopoietic Stem Cells)
• Polycythaemia Vera ( Erythroid Cells)
• Essential Thrombocythaemia (Megakaryocyte)

Question 3

What can myeloproliferative disorders transform into?

Answer 3

• Acute Myeloid Leukaemia

Question 4

What mutations are these conditions associated with?

Answer 4

• JAK2
• MPL
• CALR

Question 5

What is Myelofibrosis?

Answer 5

• This is the proliferation of the cell line which leads to fibrosis of the bone marrow
• The bone marrow is then replaced by scar tissue
• This is in response to cytokines that are released from proliferating cells
• Cytokine in particular = Fibroblast growth factor

Question 6

What are the symptoms of Myelofibrosis?

Answer 6

• Anaemia (due to production of the blood cells being affected)
• Leukopenia ( low WCC)
• Extramedullary Haematopoiesis ( Hepatomegaly and Splenomegaly - Portal Hypertension can occur and spinal cord compression)

Question 7

What are the signs and symptoms of the myeloproliferative disease?

Answer 7

• Splenomegaly
• Portal Hypertension
• Low Platelets
• Thrombosis
• Rasied RBC
• Low WBC

Question 8

What Blood results would you see with Polycythaemia Vera?

Answer 8

• Raised Hb

Question 9

What blood results would you see in Primary Thrombocythaemia?

Answer 9

• Raised Platelet Count

Question 10

What is seen in Myelofibrosis?

Answer 10

• Variable findings
• Anaemia
• Leukocytosis
• Thrombocytosis
• What would you see on the blood film in myelofibrosis (teardrop-shaped RBCs)
• Varying sizes of RBC (Poikilocytosis)
• Immature Red an

Question 11

Secondary causes of thrombocytopenia

Answer 11

• bleeding
• iron deficiency
• hyposplenism

Question 12

Management of thrombocytopenia

Answer 12

• anti-platelets to everyone
• IPSET