Acute Myeloblastic Leukaemia with Marrow Fibrosis (Malignant Myelosclerosis): Acute Leukaemia or Malignant Myelosclerosis?

Abstract

Malignant myelosclerosis was described as a rare form of acute myeloproliferative disorder characterized by minimal or absent splenomegaly, cytopenias, circulating myeloblasts and diffuse marrow fibrosis, but distinction between this entity and acute myeloblastic leukemia with marrow fibrosis is difficult. This report describes a 50 yr old man who presented with features similar to those of malignant myelosclerosis but the terminal stage was characterized by generalized lymphadenopathy, bone tenderness, marked leukocytosis and myeloblastemia. Postmortem examination showed multiple chloromas consisting of myeloblasts. Malignant myelosclerosis may be a variant of acute myeloblastic leukemia and probably should be treated similarly. A possible association between diagnostic medical irradiation and this acute myeloproliferative disorder is discussed.