Polycythaemia vera terminating in acute leukaemia. A clinical, cytogenetic and morphologic study in 8 patients...

A total of 120 patients with polycythaemia vera (PV) were observed between 1971-1976. 8 of them developed acute leukaemia (AL). The clinical course, cytogenetic and morphologic findings of these patients are described in detail. Alkylating agents were the main treatment for all patients who developed AL. 4 of them obtained alkylating drugs alone. Deaths in AL constituted 36% of the deaths observed in the groups treated with alkylating agents and 23% of the total number of deaths in these series. All patients who developed AL had active disease but they had no distinctive features predicting a malignant development. The time interval between PV diagnosis and development of AL was relatively short for those patients who were induced and maintained with alkylating agents alone. It is remarkable that 3 out of 8 patients had erythroleukaemia. Cytogenetic pretreatment studies were performed in 3 patients and all were normal. 7 patients were studied with banding techniques during the leukaemic state and all but one have shown multiple and complex abnormalities indicating several super-imposed cell lines. It seems probable that PV patients treated with alkylating agents for remission induction and maintenance may run even a greater risk for AL development than those treated with 32P.