Thymic alymphoplasia, lymphoma, and dys-gamma-globulinemia, hyper-gamma A-, normo-gamma M-, hypo-gamma G, a-gamma D-, and gamma E-globulinemia, plasmacytosis, normal delayed hypersensitivity, severe allergic reactions, and Coombs' positive anemia

• Published in: Pediatrics (Evanston) 1967-03, Vol.39 (3), p.348-362
• Main Authors: Goldman, A S, Haggard, M E, McFadden, J, Ritzmann, S E, Houston, E W, Bratcher, R L, Weiss, K G, Box, E M, Szekrenyes, J W
• Format: Article
• Language: English
• Subjects: Agammaglobulinemia - complications; Anemia - complications; Blood Protein Electrophoresis; Coombs Test; gamma-Globulins; Humans; Hypersensitivity - complications; Hypersensitivity, Delayed; Infant; Lymphocytes; Lymphoid Tissue - abnormalities; Lymphoma - complications; Male; Plasma Cells; Pneumonia, Pneumocystis - complications; Skin Tests; Thymus Gland - abnormalities
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.39.3.348

A male infant is described with thymic alymphoplasia; lymphocytopenia; lymphoid tissue hypoplasia; plasmacytosis of lymph nodes and marrow; dys-γ-globulinemia characterized by hyper-γA-, normo-γM-, hypo-γG-, and a-γD- and γE-globulinemia and decreased K- and λ-light chains with an increased K/λ 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-γ-globulinemia. The frequency of allergic disorders was increased in family members of these children.