The Management of Polycythaemia Vera

Abstract

Thrombo-embolic complications are the commonest cause of morbidity and mortality in untreated PV. Control of the PCV (<0.45) and platelet count (<400 x 10⁹/I) minimizes the risk of these complications. In addition, suppression of megakaryocytic proliferation delays myelofibrotic transformation, which is probably the ‘natural’ long-term outcome of PV. A small percentage of patients transform to acute leukaemia, particularly after myelofibrotic transformation. Leukaemic transformation is enhanced with some forms of myelosuppression, notably high accumulated doses of chlorambucil and ³²P. Venesection should be used initially to reduce the PCV and subsequently as required. The choice of myelosuppressive agent depend on the patients age. Older patients can be managed with ³²P, low-dose busulphan or pipobroman. Hydroxyurea has a role in the management of patients at all ages, although it possibly enhances the leukaemogenic risk. Therefore, agents such as interferon-α or anagrelide should be considered in younger patients.

Keywords:

• Polycythaemia vera
• treatment
• management