Abstract
Three male children in 1 family (2 siblings and 1 maternal cousin) have presented with an illness characterized by cervical adenopathy, hepatosplenomegaly, fever and subsequent development of agammaglobulinemia in the 2 survivors. Both siblings presented at age 8 with positive heterophile titers. EBV titers were not obtained. The maternal cousin became ill at age 6 months; EBV antibodies were detected on 3 occasions but the heterophile titer was negative. Peripheral WBC rose to a level of 120,000/mm3 with 90% lymphocytes, most being “atypical” in morphology. Evaluation of peripheral blood lymphocytes at the height of the illness showed 94% T-cells and 6% B-cells with normal lymphocyte transformation in response to phytohemagglutinin. Immunoglobulin analysis initially showed marked IgM elevation with normal IgA and decreased IgG. Subsequently serial analyses revealed absence of the 3 major immunoglobulins. Lymph node biopsies of the surviving sibling and cousin showed replacement of normal architecture by necrotic material. The association (x-linked) of acquired agammaglobulinemia following an illness with features of infectious mononucleosis has not been previously reported. Its pathogenesis may involve an abnormal T-cell response to EBV transformation of B-cells leading to B-cell dysfunction, lymph node necrosis, and agammaglobulinemia.
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Provisor, A., Iacuone, J., Chilcote, R. et al. ACQUIRED AGAMMAGLOBULINEMIA IN 3 RELATED MALE CHILDREN FOLLOWING AN ILLNESS WITH CLINICAL AND LABORATORY FEATURES OF INFECTIOUS MONONUCLEOSIS. Pediatr Res 8, 417 (1974). https://doi.org/10.1203/00006450-197404000-00464
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DOI: https://doi.org/10.1203/00006450-197404000-00464