Idiopathic acquired sideroblastic anemia terminating in acute myelosclerosis. 1982

W M Butler, and H G Taylor, and U Viswanathan

Acute myelosclerosis is an unusual myeloproliferative syndrome characterized by pancytopenia, absence of massive hepatosplenomegaly, and an absence of tear-drop poikilocytes. The marrow is usually fibrotic with atypical megakaryocytic and trilinear dyspoiesis. Median survival is approximately six months from onset of symptoms. The authors report a case of acute myelosclerosis that evolved in a patient two and one-half years after the onset of idiopathic acquired sideroblastic anemia. A review of the other previously reported case of this unusual transformation and comments on the pathogenesis of the transformation from idiopathic acquired sideroblastic anemia to acute myelofibrosis are included.

UI MeSH Term Description Entries
D008297 Male Males
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208 Acute Disease Disease having a short and relatively severe course. Acute Diseases,Disease, Acute,Diseases, Acute
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000756 Anemia, Sideroblastic Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow. Anemias, Sideroblastic,Sideroblastic Anemia,Sideroblastic Anemias
D055728 Primary Myelofibrosis A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone. Agnogenic Myeloid Metaplasia,Bone Marrow Fibrosis,Chronic Idiopathic Myelofibrosis,Fibrosis, Bone Marrow,Idiopathic Myelofibrosis,Myelofibrosis,Myelofibrosis With Myeloid Metaplasia,Myeloid Metaplasia,Myelosclerosis,Myelosis, Nonleukemic,Agnogenic Myeloid Metaplasias,Bone Marrow Fibroses,Fibroses, Bone Marrow,Metaplasia, Agnogenic Myeloid,Metaplasia, Myeloid,Metaplasias, Agnogenic Myeloid,Metaplasias, Myeloid,Myelofibroses,Myelofibroses, Primary,Myelofibrosis, Primary,Myeloid Metaplasia, Agnogenic,Myeloid Metaplasias,Myeloid Metaplasias, Agnogenic,Myeloscleroses,Myeloses, Nonleukemic,Nonleukemic Myeloses,Nonleukemic Myelosis,Primary Myelofibroses

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