Myeloproliferative disorders (CH) Flashcards
List examples of myeloproliferative disorders. (4)
- chronic myeloid leukaemia
- polycythaemia vera
- essential thrombocytosis
- primary myelofibrosis
What is polycythaemia vera (myeloproliferative disorder)?
Blood cancer, where bone marrow makes too many RBCs (independent of EPO)
What is essential thrombocytosis (myeloproliferative disorder)?
Chronic myeloproliferative disorder associated with sustained dysregulated megakaryocyte proliferation in the bone marrow, increasing the number of circulating platelets causing thrombocytosis
What is the average age at diagnosis of essential thrombocytosis?
50-70 years old
What mutation is found in around 50% of patients with essential thrombocytosis (also associated with polycythaemia vera)?
JAK2 mutation
What is primary myelofibrosis (myeloproliferative disorder)?
Extensive scarring in your bone marrow, leading to severe anaemia that causes weakness and fatigue
What are the clinical features of primary myelofibrosis? (5)
- severe anaemia –> weakness + fatigue
- massive splenomegaly
- pancytopenia
- dry tap - failure of BM aspirate
- tear drop poikilocytes (dacrocytes) on blood film - flat, elongated RBCs
What would you see on bloods in myelofibrosis? (4)
- anaemia
- thrombocytosis
- raised LDH
- raised uric acid
How do we treat primary myelofibrosis? (3)
- asymptomatic - no Rx required
- symptomatic - haematopoietic stem cell transplant
- JAK2 inhibitor (Ruxolitinib) can be used to manage splenomegaly and B symptoms, but does not prolong life
What are the main conditions with massive splenomegaly? (2)
- chronic myeloid leukaemia
- myelofibrosis
What are the general clinical features of myeloproliferative disorders? (4)
- erythromelalgia - burning pain of extremities, increasing with heat and improving with cold
- splenomegaly
- arterial and venous thrombosis
- bleeding
What will FBC show in myeloproliferative disorders?
Platelet count >450x10^9/L
What specific testing can you do for myeloproliferative disorders?
JAK2 mutation testing
How do we manage essential thrombocytosis (myeloproliferative disorder)?
- hydroxycarbamide - to reduce platelet count
- emergency - plateletpheresis
- antiplatelet therapy (aspirin) to reduce thrombotic risk
What are some complications of myeloproliferative disorders? (3)
- thrombosis
- bleeding
- intrauterine death of foetus
