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Angioimmunoblastic lymphadenopathy: Clinical spectrum of disease
Author(s) -
Schauer Peter K.,
Straus David J.,
Clarkson Bayard D.,
Bagley Charles M.,
Rudolph Robert H.,
Huff John,
Glucksburg Harold,
McCracken Joseph D.,
Bauermeister Donald E.
Publication year - 1981
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19811201)48:11<2493::aid-cncr2820481124>3.0.co;2-t
Subject(s) - medicine , hepatosplenomegaly , generalized lymphadenopathy , rash , prednisone , inguinal lymphadenopathy , autoimmune hemolytic anemia , immunosuppression , anemia , axillary lymphadenopathy , melena , surgery , sepsis , hypergammaglobulinemia , dermatology , lymphoma , disease , biopsy , cancer , breast cancer
The clinical features of 13 patients with angioimmunoblastic lymphadenopathy were analyzed to determine prognostic factors and response to therapy. Eleven patients presented with sudden onset of fever, weight loss, generalized lymphadenopathy, and hepatosplenomegaly. Laboratory features included autoimmune hemolytic anemia and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in six cases and skin rash in four. Two patients had localized lymphadenopathy without systemic symptoms. Both are alive at 5.5 and 2.5 years, respectively, after diagnosis, although the latter patient has required intermittent prednisone for recurrent lymphadenopathy. An additional patient is alive on treatment four months following diagnosis. The remaining ten have died, nine of sepsis and one of cerebral hemorrhage. The immunosuppression and myelosuppression of combination chemotherapy may have hastened their deaths. An individualized, conservative treatment approach is recommended.