Flashcards in Chronic Myeloproliferative Disorders and Chronic Myeloid Leukaemia Deck (25)
What are chronic myeloproliferative disorders?
Chronic overproduction of myeloid cells
What is polycythaemia vera?
Increased number of RBCs (+/- neutrophils; +/- platelets)
What is essential thrombocythaemia?
Increased number of platelets
What is myelofibrosis?
Variable cytopenias with LARGE spleen and fibrosis(distinguished from other causes of splenomegaly)
What are the symptoms of polycythaemia vera?
Plethoric face (red face)
Gangrene of toes/fingers
What are the signs of polycythaemia vera?
Retinal veins engorgment
What is the diagnosis of polycythaemia vera?
Persistent increase in Hb > 0.5 ratio of constituents (RBC, buffy coat and plasma)
When diagnosing polycythaemia vera, what questions do we need to consider?
Is it relative or absolute polycythaemia (e.g. is it just reduced amount of plasma (dehydration) or increased RBCs)
Primary vs secondary (is something else causing increased RBCs (e.g. EPO at altitude)
What investigative test should be performed?
FBC and ferritin (full blood count and iron)
UE/LFT (liver and kidney function tests)
Name some causes for secondary polycythaemia?
EPO production tumours
Chronic lung disease
What genetic mutations are associated with polycythaemia vera?
What is the treatment for polycythaemia vera?
Venesection - extract blood with aim of maintain hematocrits level below 0.45
Aspirin - COX2 inhibitor - blood thinner
What are the causes of secondary 'reactive' thrombocytocis?
What is wrong with primary thrombocytosis?
What is the definition of thrombocytosis?
Persistent platelet above 450 X 10^9
What are the mutation associated with thrombocytosis?
How is thrombocytosis treated?
Assess thrombotic risk
Antiplatelt treatment i.e. aspirin
Cytoreduction if high risk e.g. hydroxycarbomide, interferon
What are both patients with thrombocytosis or polycythaemia at risk of developing?
Acute myeloid leukaemia
What are the major presenting features of myelofibrosis?
Splenomegaly - due to bone marrow fibrosis (spleen take over function)
B cell symptoms - night sweats, fever, weight loss
What are the diagnostic features of myelofibrosis?
Bone marrow results
What are the causes of splenomegaly? (CHICAGO)
Congestion e.g. liver
Other e.g. amyloid
What is the treatment for myelofibrosis?
-Supportive treatment e.g. EPO
-Bone marrow transplant
Poor prognosis - 5 yrs
What are the main characteristics of chronic myeloid leukaemia?
What is the main cause of chronic myeloid leukaemia?
Philadelphia chromosome (translocation 9:22)