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
1967; American Academy of Pediatrics; Volume: 39; Issue: 3 Linguagem: Inglês
10.1542/peds.39.3.348
ISSN1098-4275
AutoresArmond S. Goldman, Mary Ellen Haggard, John Mcfadden, Stephan E. Ritzmann, Elsie W. Houston, Raymond L. Bratcher, Kurt G. Weiss, Edith M. Box, Julius W. Szekrenyes,
Tópico(s)Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
ResumoA 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.
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