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Leukemia in patients with acquired idiopathic sideroblastic anemia: An evaluation of prognostic indicators
Author(s) -
Lewy Robert Ira,
Kansu Emin,
Gabuzda Thomas
Publication year - 1979
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830060404
Subject(s) - medicine , mean corpuscular volume , chronic myelomonocytic leukemia , hemochromatosis , anemia , leukemia , neutropenia , gastroenterology , myelodysplastic syndromes , ineffective erythropoiesis , surgery , sideroblastic anemia , erythropoiesis , hematocrit , bone marrow , chemotherapy
The initial clinical and laboratory data of 25 patients with acquired idiopathic sideroblastic anemia (AISA) were anlayzed. Criteria for accepting the diagnosis were hyperferremia, ringed marrow sideroblasts, ineffective erythropoiesis, and exclusion of associated hematologic disorders. The findings of a mean age at onset of 70 years, increased mean corpuscular volume, relative neutropenia, and occasional splenomegaly at diagnosis corresponded with previous reports. During the followup for a median period of 32 months, 6 patients (25%) transformed to acute myelogenous or myelomonocytic leukemia after widely variable intervals. The initial data base of these patients was compared to that of the remaining 19 patients in order to isolate predictive features. Only a lesser degree of hyperferremia (P < 0.001) made the group going on to leukemia distinctive. The median survival of these patients was 20 months. The median survival of 19 patients not developing leukemia was 72 months for males and 42 months for females. Hemochromatosis was diagnosed in four patients and was a primary or associated cause of death in three. Analysis of the transfusion history suggested that intrinsic iron leading was a major factor in these patients. We conclude that leukemic transformation in AISA is a common, poorly predictable event which required lengthy followup for detection. Hemochromatosis in AISA occurs frequently and shortens the median survival.