Hypogammaglobulinemia with sarcoidlike granulomas

A 12-year-old girl had sarcoidlike syndrome and hypogammaglobulinemia. Pancytopenia and hepatosplenomegaly were noted at age 4 years. Histopathologic study showed typical sarcoidlike granulomas. Chronic lung disease, along with recurrent infections, developed. Immunologic studies revealed common variable hypogammaglobulinemia, abnormal cellular immune functions, and decreased C4 levels. An immunoregulatory defect is suggested as the pathogenesis of this immunodeficiency syndrome, with multisystem sarcoidlike granulomas, hypersplenism, pulmonary disease, and abnormal cellular and humoral immunity.