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Epidemiology of aplastic anemia in France: a prospective multicentric
study. The French Cooperative Group for Epidemiological Study of Aplastic
Anemia
JY Mary, E Baumelou and M Guiguet
INSERM U263, Unite de Recherches Biomathematiques et Biostatistiques,
Universite Paris, France.
Incidence rates of aplastic anemia (AA) are rare among defined populations.
Since June, 1984, a cooperative group, including 83 University medical
centers throughout metropolitan France, prospectively recorded new cases of
AA and followed them up. Inclusion criteria were: at least two depressed
blood cell lineages (hemoglobin less than or equal to 10 g/100 mL and
reticulocytes less than or equal to 50 x 10(9)/L, granulocytes less than or
equal to 1.5 x 10(9)/L, platelets less than or equal to 100 x 10(9)/L) and
a bone marrow biopsy compatible with the disease. Between May, 1984, and
April, 1987, 292 cases were recorded. After exclusion of constitutional
disease, 27 patients did not satisfy the inclusion criteria with relation
to either bone marrow or blood evaluations and seven patients were
initially misdiagnosed (shown in the follow-up), leaving 250 confirmed AA
cases in the register. The annual incidence in France appeared to be about
1.5 per million inhabitants. The sex ratio of AA cases was similar to that
of the population. In men, two peaks of incidence were observed: one
between 15 and 30 years and one after 60 years. In women, the only peak was
observed after 60 years. An excess of cases was observed in small towns but
not in rural areas. About two of every three cases had severe AA, with a
possible excess in younger cases. Based on a minimum follow-up of 1 year
for 238 patients, the fatality rate was estimated at 17% at 3 months after
diagnosis and at 34% at 1 year. Among 243 suspected etiologies reported by
the physicians, 74% were declared idiopathic, 13% presumably associated to
drug toxicity, and 5% related to hepatitis. AA appears to be a rare and
severe disease in metropolitan France, often of unknown origin, a fact that
emphasizes the necessity of controlled etiologic studies.
Volume 75,
Issue 8,
pp. 1646-1653,
04/15/1990
Copyright © 1990 by The American Society of Hematology
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