Clinical course and survival after elective splenectomy in 19 patients with primary myelofibrosis.

Nineteen patients with primary myelofibrosis and myeloid metaplasia (MMM), who fulfilled well-defined criteria, underwent elective splenectomy as soon as the diagnosis was confirmed. Nine patients developed postoperative complications such as intra-abdominal bleeding (three), rupture of the inferior vena cava (one), ascites (two), Australia antigen-positive hepatitis (three), mesenteric artery thrombosis (one) and pneumonia (one). One patient died within a month of the operation due to secondary intra-abdominal infection. The mean age of the patients at splenectomy was 56 years and the mean duration of their disease 2.4 years. The median actuarial survival after operation was 51 months. Although the series of patients is small, it seems that splenectomy did not have an adverse effect on life expectancy. The haematological status and the quality of life improved after splenectomy in 17 of 19 patients. The results warrant a further trial with elective splenectomy in an early stage of MMM.