[Angioimmunoblastic lymphadenopathy. 8 cases and review of the literature].
Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
D000328:Adult; D000368:Aged; D005260:Female; D005334:Fever / Q000209:etiology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications; D007119:Immunoblastic Lymphadenopathy / Q000473:pathology*; D007075:Immunoglobulin M; D008223:Lymphoma / Q000150:complications; D008297:Male; D008875:Middle Aged; D012877:Skin Manifestations; D013163:Splenomegaly / Q000209:etiology; D013587:Syphilis / Q000150:complications; D013789:Thalassemia / Q000150:complications
D007075:Immunoglobulin M
Lymphadénopathies angio-immunoblastiques. Observation de 8 cas et reveu de la littérature.