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Transcript of Myeloproliferative Disorders
Increase in red cell volume due to a CLONAL malignancy of a marrow stem cell
May also have ↑neutrophils and platelets
Polycythaemia Rubra Vera
Leads to cell proliferation as JAK2 protein is constitutively activated
Clinical Features due to
(HYPERmetabolism & Haemolysis)
Headaches & dizziness
Pruritis (HOT BATH!)
Gout (↑ urate)
↑ red cell count
↑ packed cell volume
Low dose aspirin
Cytotoxic drugs e.g. hyrdoxycarbamide if symptomatic splenomegaly
Radioactive phosphorus if over 70 - risk of leukaemia
Most people remain well!
Thrombosis / haemorrhage - main complications
30% progress to myelofibrosis
5% progress to acute leukaemia
CLONAL proliferation of megakaryocytes leads to ↑ PLATELETS (thrombocytosis) with impaired function
~50% have JAK2
FBCs (+ repeat)
Platelets persistently over 600x10^9/L
Exclude other causes e.g. reactive due to bleeding, infection, malignancy, chronic inflammation
ESR, CRP, Fibrinogen - NORMAL
Low dose ASPIRIN
Plts over 1500
Causes myelosuppression so reduces plts!
Can progress to...
Marrow SCARRING due to hyperplasia of megakaryocytes which cause ↑ production of platelet derived growth factor.
Myeloid metaplasia -->
JAK2 - 50-60% of patients
Plts initially high then thrombocytopenia
Low red cell count
WCC - high or low!
Bone marrow aspirate - DRY
Bone marrow trephine - diagnostic - shows fibrosis
Myeloblative bone marrow transplant (allogeneic stem cell transplant)
50-65 year olds
Non-myeloblative bone marrow transplant
Spleen reduction - medically (e.g. thalidomide) or surgically (splenectomy)
Marrow support - platelet / blood transfusions
Stem cell transplant can be curative in younger patients.
Prognosis if no transplant - median survival of 5 years
Trial - JAK2 inhibitors
Abdominal discomfort - big spleen!
BONE MARROW FAILURE
Uncontrolled clonal proliferation of myeloid precursors which still have their ability to differentiate intact - malignancy of granulocytes
Mostly 40-60 yr olds but can be younger
Chronic - few symptoms - can last years!
Accelerated - increasing symptoms and splenic size - symptoms from cellular accumulation
Acute - blast transformation; symptoms from marrow failure
↑↑↑ WCC (often >100x10^9 / L)
Neutrophils, basophils, eosinophils, myelocytes
↓ Hb (or normal) / Plts variable
Urate and B12 ↑
Bone marrow - hypercellular
Cytogenetics - look for Philly!
Molecular test on blood - BCR-ABL FISH
Abdominal discomfort - SPLENOMEGALY
tYROSINE kINASE iNHIBITOR
UNCONTROLLED PROLIFERATION OF ONE OR MORE OF THE MYELOID STEM CELL LINE
Cells look quite NORMAL
Can progress to acute leukaemia - cells look ABNORMAL