[Myeloproliferative diseases]

Abstract

Myeloproliferative diseases include primary polycythemia, primary thrombocytosis, primary myelofibrosis and chronic myeloid leukaemia. The average survival of patients with the former two diagnoses is more than 10 years, in the subsequent two it is only 5 years. Standard treatment of polycythemia still remains phlebotomy, only in patients with associated thrombocythemia and complications caused by it cytoreduction treatment is recommended (hydroxyurea, busulphan or interferon alpha). Patients with primary thrombocythemia due to hypofunctional thrombocytes suffer frequently from haemorrhagic and thrombotic complications. The objective of treatment is therefore to achieve a normal number of thrombocytes. Medicamentous procedures are the same as in primary polycythemia. In primary myelofibrosis and chronic myeloid leukaemia it is also necessary to reduce the pathological population in bone marrow by the above mentioned therapeutic procedures. Contrary to the former two diagnoses, the survival of patients with primary myelofibrosis is shorter and the patients need more supportive treatment. In chronic myeloid leukaemia also transplantation treatment can be used, if a suitable donor is available. The decision between classical and transplantation treatment must be made soon after establishment of the diagnosis. The objective of the following paper is to inform readers on the clinical course of different diseases, on differential diagnostic problems and changes in therapeutic procedure which have developed in recent years.