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Treatment of Erythrocytosis in Polycythemia Vera with Dapson

Treatment of Erythrocytosis in Polycythemia Vera with Dapson

Blood 100(11): Abstract No 4941, November 16

Background: The correction of erythrocytosis in patients with polycythaemia vera (PV) is essential to prevent thrombotic complications. Usually this is done by repeated phlebotomies. However this strategy is hampered by an increased the risk of progressive marrow fibrosis. Alternatively, myelosuppressive therapy carries an increased risk of leukemic transformation. Recently we observed an immediate and sustained decrease of the hematocrit upon the administration of dapsone in a patient with PV poorly responding to repeated phlebotomies. To further assess the efficacy of dapsone on the erythrocytosis we treated 12 PV patients with dapsone 50-200 mg daily. They received no other treatment. Results: The median Hb decreased from 10,4 to 8,6 mmol/l, the Ht from 0,52 to 0,43. In all but three patients the hematocrit could be brought below the value of 0,45 in man or 0,42 in women. All patients developed laboratory symptoms of chronic hemolysis: the median reticulocyte count rose from 2,2 to 4,1%, the LDH from 175 to 280 u/l and the bilirubin from 11 to 22 mmol/l. The haptoglobin decreased from median 0,8 to 0,0 g/l. Therefore low grade chronic hemolysis induced by dapsone appears to be the mode of action in the treatment of erythrocytosis. Side effects were noted in 6 patients consisting of headache (n=3), fatigue (n=2) and dizziness (n=1). Only in one patient the side effect was reason to stop treatment. Side effects might be related to the methemoglobinaemia, the median concentration rose from 0,6 to 4,6% (range 2,1-12,1%). Four patients continue dapsone as single therapy for 11, 29, 34 and 117 months respectively with adequate control of their polycythemia vera. Two patients continued dapsone but additional therapy had to be given to control the erythrocytosis. In 6 patients the dapsone was stopped after a median of 7 months (range 1-17 months) because of : increasing splenomegaly (n=1), leuko- and trombocytosis (n=2), anaemia (n=1) and fatigue (n=1). Conclusion: Dapsone is a valuable drug in the control of erythrocytosis in polycythaemia vera. It is well tolerated and lacks the untoward side effects of prolonged phlebotomies or myelosuppressive therapy.

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