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Studies of a familial platelet disorder
SB Dowton, D Beardsley, D Jamison, S Blattner and FP Li
At least 22 members of a large kindred have a bleeding tendency resulting
from an autosomal dominant disorder of platelet production and function.
Phenotypic manifestations include mild to moderate thrombocytopenia,
bleeding time prolongation, and abnormal platelet aggregation. Platelet
survival time is normal. The platelet disorder in this family appears to
differ from known hereditary thrombocytopenic or thrombocytopathic
syndromes and may represent a new genetic disease. Six family members
reportedly developed hematologic neoplasms: acute monocytic leukemia nine
years after treatment for congenital neuroblastoma; lymphosarcoma at age 10
years; myeloid leukemia at age 23 years; acute myelocytic leukemia at age
62 years; leukemia of unknown type at age 48 years; and lymphocytic
lymphoma at age 52 years.
Volume 65,
Issue 3,
pp. 557-563,
03/01/1985
Copyright © 1985 by The American Society of Hematology
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