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Natural history of idiopathic refractory sideroblastic anemia
M Cazzola, G Barosi, PG Gobbi, R Invernizzi, A Riccardi and E Ascari
Department of Internal Medicine and Medical Therapy, University of Pavia
School of Medicine, Italy.
We analyzed the natural history of idiopathic refractory sideroblastic
anemia (IRSA) in 37 patients studied between 1969 and 1986. Although
erythroid abnormalities were prominent in all, 12 patients also showed
involvement of the granulocytic and/or megakaryocytic cell lines, and
nonrandom chromosomal aberrations were observed in five of 23 patients
studied for such defects. Measurements of erythroid marrow function showed
in most cases erythroid expansion with ineffective erythropoiesis. In seven
patients, however, the erythroid activity was found to be inappropriately
low for the degree of anemia. Transfusion dependence occurred in 26 of 37
cases. Iron overload was a common feature at presentation but produced
clinical manifestations of hemochromatosis only in those patients who
subsequently had a regular need for blood transfusions. Five patients
progressed to bone marrow failure, and another five patients (two of whom
had monosomy 7) evolved into acute nonlymphocytic leukemia (ANLL). The
median survival was 72 months, with a high transfusion requirement,
multilineage defects, and inappropriately low erythroid proliferation being
associated with a poor prognosis. The most common causes of death were
complications of iron overload and evolution into ANLL. We conclude that
(a) the natural history of IRSA is characterized by an initial phase of
erythroid hyperplasia and ineffective erythropoiesis, which is usually
stable for many years but in a subset of patients may be followed by a
phase of marrow failure with or without the later emergence of leukemic
blasts; (b) peripheral blood counts, measurement of erythroid marrow
function, and chromosomal analysis are useful for identifying subjects at
risk of evolution into marrow failure or ANLL; and (c) IRSA patients with
no need for blood transfusions are very likely to be long survivors,
whereas those who become transfusion dependent are at risk of death from
the complications of secondary hemochromatosis.
Volume 71,
Issue 2,
pp. 305-312,
02/01/1988
Copyright © 1988 by The American Society of Hematology
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