Chronic idiopathic thrombocytopenic purpura. A familial immunodeficiency syndrome?

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Abstract
Three children with chronic idiopathic thrombocytopenic purpura (ITP) and their immediate families were studied. All 3 patients and 10/13 family members manifested at least 1 immunologic defect, e.g., decreased numbers of T [thymus-derived] lymphocytes (1/3, 5/13), diminished in vitro response to phytohemagglutinin (2/3, 6/13), dysgammaglobulinemia (2/3, 4/13), altered autoantibodies (1/3, 5/13), and decreased serum properdin levels (3/3, 2/13). One parent and 2 asymptomatic siblings of 2 of the propositi also had shortened platelet life spans with normal platelet counts. The HLA antigens A3 and B7 were identified in all 3 families. There also appeared to be an association between a familial haplotype and the immunologic defects. Chronic ITP appears to occur in families with underlying immunologic defects, which are genetically related.