Myeloproliferative neoplasms Flashcards
What is polycythaemia vera?
Excess of RBCs and platelets
What are the clinical features of polycythaemia vera?
Aquagenic itch Erythromyalgia- rare but classic Thrombosis Headache, fatigue, hepatosplenomegaly Gouty arthritis
What investigations should be done for polycythaemia vera?
RBC, uric acid
JAK2 kinase
What is the treatment of polycythaemia vera?
Venesection
Aspirin
Hydroxucarbamide- stroke prevention
JAK2 inhibitor- if systemic symptoms
What are the complications of polycythaemia vera?
Stroke
Thrombosis
Bone marrow failure
Progression to AML
Wat is essential thrombocythaemia?
High platelets and JAK2 mutation
What are the clinical features of ET?
Arterial and venous thrombosis Bleeding Gout Headache Mild splenomegaly
How is ET managed
Aspirin if low risk
Hydroxycarbamide or anagrelide if higher risk
Whatare the complications of ET?
Progression to myelofibrosis/AML
What is idiopathic myelofibrosis?
Fibrosis of bone marrow
What are the features of idiopathic myelofibrosis?
Bone pain- due to fibrosis
Infection, bruising/bleeding, anaemia due to failure
Hepatosplenomegaly
Gout
What investigations are done for idiopathic myelofibrosis?
FBC- pancytopenia i.e. everything low
Bone marrow biopsy
How is idiopathic myelofibrosis managed?
Mainly supportive
Allogenic stem cell transplant curative
