Autoimmune Hemolytic Anemia in a Child with Congenital X-Linked Hypogammaglobulinemia

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Abstract
In a six-year-old boy with congenital sex-linked hypogammaglobulinemia, Coombs-test positive autoimmune hemolytic anemia developed despite profound deficiencies of immunoglobulins. Direct and indirect Coombs tests using anti IgG and IgM serums were positive. The eluted antibody was an IgG globulin with both kappa and lambda light-chain determinants. The antibody had specificity for the public Rh antigen designated LW. During two years of observation the patient responded well to corticosteroids, but relapsed when these were discontinued. Azathioprine therapy did not change the serologic findings.