Thrombocytopenia With Elevated Serum Iga And Renal Disease

Gutenberger et al. (1970) described a kindred in which 10 males and 2 females had thrombocytopenia apparently as a result of reduced platelet production. Eight of the thrombocytopenic persons had elevated IgA levels in the serum. Renal biopsy in 3 thrombocytopenic brothers with hematuria showed varying degrees of glomerulonephritis. The 2 thrombocytopenic women were mothers of affected sons. One of these 2 women also had elevated IgA. The authors concluded that the disorder is X-linked and probably distinct from the Wiskott-Aldrich syndrome (301000) and from 'simple' X-linked thrombocytopenia (313900).