THYMIC ALYMPHOPLASIA, LYMPHOMA, AND DYS-γ-GLOBULINEMIA. HYPER-γA-, NORMO-γM-, HYPO-γG, A-γD-, AND γE-GLOBULINEMIA, PLASMACYTOSIS, NORMAL DELAYED HYPERSENSITIVITY, SEVERE ALLERGIC REACTIONS, AND COOMBS' POSITIVE ANEMIA
- 1 March 1967
- journal article
- Published by American Academy of Pediatrics (AAP)
- Vol. 39 (3) , 348-362
- https://doi.org/10.1542/peds.39.3.348
Abstract
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.Keywords
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