Treatment of primary proliferative polycythaemia by venesection and low dose busulphan: retrospective study from one...

Sixty-five patients with primary proliferative polycythaemia (polycythaemia rubra vera) were followed during the period 1962-83 and analysed retrospectively. Primary control of PCV was by venesection only with low dose busulphan solely as required to keep the platelet count below 400 X 10(9)/l. Median survival was 11.1 years from diagnosis which is equal to or marginally better than with other reported regimens. Vascular causes of death were only a little higher than expected in a comparable normal population. Only deaths from acute leukaemia and myelofibrosis were significantly increased above the normal population incidence. There was no evidence to suggest that these transformations were busulphan induced. Analysis of the incidence of occlusive vascular lesions lends support to an earlier recommendation that the PCV level be maintained below 0.45. No support was found for the possible disadvantages of a predominantly venesection regimen, such as iron deficiency and reactive thrombocytosis. The case is put for this use of low dose busulphan. The data presented would warrant the future inclusion of this therapeutic regime as one limb of a controlled trial.