[Acquired immunodeficiency syndrome with chronic granulomatous inflammation. Clinically definable special form of the variable immunodeficiency syndrome].
Five cases (3 men, 2 women) of late-onset variable immunodeficiency syndrome (CVID), characterized by similar clinical and immunological findings as well as histological demonstration of chronic granulomatous infection, are reported. All patients had frequent attacks of respiratory infections with recurrent bronchitis and pneumonia. In addition to predominating basally localized streaky-nodular lung changes all patients had hepatosplenomegaly and granulomatous infections of other organs. Immunologically, marked hypogammaglobulinaemia of all Ig classes, lymphopenia, and absence of terminal B-cell maturation were predominant. In-vitro tests under pokeweed-mitogen failed to demonstrate terminal plasma-cell differentiation of B-lymphocytes and thus Ig synthesis. Without pokeweed-mitogen there were largely nonsecretory B-blasts with abnormal granulated cytoplasmic Ig formation. Skin testing with Multitest application revealed almost complete anergy, both in the Arthus (24 h) and the late reactions (48 and 72 h). Nonetheless, T-cell reaction in-vitro was much less affected than B-cell function. "Natural killing" and antibody-dependent cytotoxicity were normal or slightly increased.
D000163:Acquired Immunodeficiency Syndrome / Q000276:immunology* / Q000473:pathology; D000293:Adolescent; D000328:Adult; D000920:Antibody-Dependent Cell Cytotoxicity; D001402:B-Lymphocytes / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007428:Intradermal Tests; D007694:Killer Cells, Natural / Q000276:immunology; D008168:Lung / Q000473:pathology; D008213:Lymphocyte Activation; D008297:Male; D012397:Rosette Formation
Erworbenes Antikörpermangelsyndrom mit chronisch-granulomatöser Entzündung. Klinisch abgrenzbare Sonderform des variablen Immundefektsyndroms.