IDIOPATHIC MYELOFIBROSIS (AGNOGENIC MYELOID METAPLASIA) - CLINICOPATHOLOGICAL ANALYSIS OF 32 PATIENTS

The prognostic value of clinicopathological parameters, recorded at di agnosis, in idiopathic myelofibrosis (IMF) was retrospectively analyze d in a consecutive series of 32 patients followed for a minimum of 5 a nd maximum of 134 months in the period 1983-1994. Of the 32 patients, 18 were males and 14 were females. The mean age was 59.8 years (S.D., 10.4; range, 44-78 years). At the time of closure of the study (May 19 95), 10 patients were dead, 18 were alive, and four were lost to follo w up. The mean survival was 41 months (range 2-130 months). Univariate analysis showed that the following features were associated with a si gnificantly shorter survival: (1) anemia (hematocrit <30%), (2) platel et count <100,000/mm(3). Age, sex, size of spleen and liver, WBC count , increased reticulin and collagen fibrosis were not of prognostic sig nificance. Clinicopathological correlation was found between the sinus al hemopoiesis and the number of WBC precursors in peripheral blood, a nd between WBC precursors and spleen size. Medullary fibrosis was foun d to be associated with striking predominance of large and very mature megakaryocytes. Erythroid hyperplasia in the bone marrow correlated n egatively with WBC precursors and spleen and liver size, and positivel y with reticulocyte count. (C) 1997 Elsevier Science Ltd.