The use of aspirin in polycythaemiavera and primary thrombocythaemia

In polycythaemiavera (PV; polycythaemiarubravera, primary proliferative polycythaemia) and primary thrombocythaemia (PT; essential thrombocythaemia), occlusive complications in the microvasculature and larger vessels are a significant cause of morbidity and mortality. Central to the pathogenesis of these complications are the quantitative and qualitative platelet changes present in these myeloproliferative disorders. Aspirin irreversibly inactivates cyclo-oxygenase in platelets. This leads to a reduced production of platelet thromboxane A2 which has vasoconstricting and platelet aggregatory properties. In haematologically normal individuals, aspirin has been shown to reduce thrombo-embolic complications in populations at risk of these events. In PV and PT, aspirin has been shown to specifically eliminate the micro-circulatory and vasomotor manifestations and there is some evidence of a reduction in larger vessel occlusion. Low-dose aspirin has been shown to substantially reduce the raised thromboxane A2 production of platelets in PV and PT patients. The incidence of haemorrhagic side-effects of aspirin are minimized by the use of low doses. Haemorrhagic events are particularly found in patients with platelet counts> 1000 x 109/1 and these events are enhanced by aspirin therapy in these patients. Aspirin should be used with caution in patients with dyspeptic symptoms or a history of peptic ulceration or bronchospasm. Precise PCV control (< 0.45) and cytoreduction (platelets < 400 × 109/1) should be used in patients with PV to minimize the vascular occlusion risk but routine cytoreduction is proposed only for those at particular risk of vascular occlusion in PT. In the acute presentation of patients with vascular occlusion, cytoreduction and an aspirin dose of 300 mg a day is proposed, reducing to 75 mg a day with the control of symptoms and signs, while 75 mg a day may play a role as prophylactic therapy in the prevention of thrombosis. However, there are no prospective studies in PT to demonstrate the benefit/risk profile and to confirm these recommendations, while a randomized prospective placebo-controlled study of low-dose aspirin in PV has only recently been initiated.