Angiofollicular and plasmacytic polyadenopathy: a pseudotumourous syndrome with dysimmunity.
Two cases are reported of an apparently distinct type of immune disorder. Beginning with mild anaemia and widespread massive lymphadenopathy, the disease progressed to a fatal autoimmune type haemolytic anaemia. Serum investigation showed polyclonal hypergamma-globulinaemia and some autoantibodies. Repeat lymph node biopsies in each case showed hyperplasia within B lymphocyte territory (follicular hyperplasia and polyclonal plasmacytosis with IgG predominance) and atrophy of T dependent areas. Dilatation of lymph sinuses, vascular proliferation, and sclerosis were striking features. This appears to be a new entity, and reasons are given for separating this disease from other pseudotumourous lymph node disorders associated with dysimmunity.
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D007074:Immunoglobulin G / Q000032:analysis*; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008206:Lymphatic Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D013577:Syndrome
D007074:Immunoglobulin G