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57 Autoimmune Hemolytic Anemia in a Patient with Congenital Hypogammaglobulinemia


Since the clinical findings of hypogammaglobulinemia (HGG) are explained by a deficient synthesis of circulating antibody, the development in such a patient of autoimmune hemolytic anemia (AHA), due to excessive production of a specific abnormal antibody is of interest. A six-year-old boy with HGG, of the sex linked variety, developed Coombs' positive AHA despite profound deficiencies of immunoglobulins (IgG 100, IgM 18, IgA 0 mg%). He had never received transfusion and penicillin had not been given for 3 months. Lymph node biopsy did not show malignant changes and cytomegalic virus could not be cultured. Direct and indirect Coombs' tests, using gost anti-human IgG and IgM sera, were positive. Eluted antibody was an IgG globulin. The serum reacted with all cells of a large panel but tests by Ortho Labs showed specificity against the public Rh antigen designated LW. The commercial gamma globulin used for therapy had no anti RBC activity. He has responded well to corticosteroids for 6 months, but has relapsed twice when this was discontinued. Although three adults with acquired HGG have developed AHA, this case represents the first instance in a patient with congenital HGG. All of the adults have had lymphoma or leukemia. Possibilities to explain this combination include graft vs host reaction with secondary ‘runt’ disease. Although unlikely in view of the patients age and lack of blood transfusions, investigations to examine this possibility are in process. Alternatively lymphoma may be present, and, if this proves true, speculations both on the nature of lymphoma and hypogammaglobulinemia are possible. (SPR)

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Pearson, H., Robbins, J. & Skinner, R. 57 Autoimmune Hemolytic Anemia in a Patient with Congenital Hypogammaglobulinemia. Pediatr Res 1, 215 (1967).

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