Acquired pancytopenia in relatives of patients with aplastic anaemia. 1980

D T Sleijfer, and N H Mulder, and H O Nieweg, and G J Anders, and W L Gouw

We studied relatives of adult patients with acquired aplastic anaemia. Eight patients were found to have 11 family members with peripheral blood pancytopenia. Six of the 11 affected relatives had diminished and four normal cellularity and one had hypercellularity of the bone marrow. Thus, 19 persons in these eight families were affected. In two families, father and son were affected, in four families brother and/or sister, in one family a brother and an aunt and in one a nephew of the index patient. None of the patients or family members had congenital defects. All patients were diagnosed at an adult age and, furthermore, also the mode of inheritance in some of the families seems to exclude Fanconi's syndrome. It is concluded that relatives of patients with aplastic anaemia should be screened for manifestations of this syndrome of familial acquired blood pancytopenias.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010198 Pancytopenia Deficiency of all three cell elements of the blood, erythrocytes, leukocytes and platelets. Pancytopenias
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000741 Anemia, Aplastic A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements. Anemia, Hypoplastic,Aplastic Anaemia,Aplastic Anemia,Anaemia, Aplastic,Aplastic Anaemias,Aplastic Anemias,Hypoplastic Anemia,Hypoplastic Anemias
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective

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