PEDIATRICS Vol. 39 No. 3 March 1967, pp. 348-362
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THYMIC ALYMPHOPLASIA, LYMPHOMA, AND DYS-ggr-GLOBULINEMIA. HYPER-ggrA-, NORMO-ggrM-, HYPO-ggrG, A-ggrD-, AND ggrE-GLOBULINEMIA, PLASMACYTOSIS, NORMAL DELAYED HYPERSENSITIVITY, SEVERE ALLERGIC REACTIONS, AND COOMBS' POSITIVE ANEMIA

Armond S. Goldman M.D.1, Mary E. Haggard M.D.1, John McFadden M.D.1, Stephan E. Ritzmann M.D.1, Elsie W. Houston M.Sc.1, Raymond L. Bratcher B.A.1, Kurt G. Weiss Ph.D.1, Edith M. Box Ph.D.1, and Julius W. Szekrenyes M.D.1

1 The Departments of Pediatrics, Internal Medicine, Pathology and Microbiology; Pediatric Immunology and Hematology Research Laboratories and the Immunohematology Laboratory; University of Texas Medical Branch, Galveston, Texas

A male infant is described with thymic alymphoplasia; lymphocytopenia; lymphoid tissue hypoplasia; plasmacytosis of lymph nodes and marrow; dys-ggr-globulinemia characterized by hyper-ggrA-, normo-ggrM-, hypo-ggrG-, and a-ggrD- and ggrE-globulinemia and decreased K- and lgr-light chains with an increased K/lgr light chain ratio; deficient formation of certain hemagglutinin, precipitin and neutralizing antibodies; the presence of skin-sensitizing, heat-labile reaginic antibodies and severe allergic reactions; normal delayed hypersensitivity; and a Coombs' positive anemia. Subsequently, a lymphoma involving bones, liver, spleen, lung, and kidney developed and terminally Pneumocystis carinii pneumonia appeared.

A male sibling who died at age 11 months manifested thymic aplasia, lymphoid tissue hypoplasia, pancytopenia and hypo-ggr-globulinemia. The frequency of allergic disorders was increased in family members of these children.

Submitted on July 11, 1966
Accepted on October 10, 1966