ANGIO-IMMUNOBLASTIC LYMPHADENOPATHY - REPORT OF 10 CASES AND REVIEW OF THE LITERATURE

  • 1 January 1979
    • journal article
    • research article
    • Vol. 48  (189) , 151-177
Abstract
Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknown etiology and pathogenesis. It has features of hyperimmunity, immune deficiency and can behave like a malignant lymphoma. An underlying abnormality of T [thymus derived] cell regulatory function was proposed. Ten patients with AIL were studied and 200 cases from the literature were reviewed. The following typical features were described in a previous retrospective series: constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin eruptions, hypergammaglobulinemia and characteristic lymph node histology were observed. Four patients had edema with ascites or pleural effusions associated with hyponatremia and hypoalbuminemia. In this paper, low free thyroxine indices were observed in 3 patients with elevated TSH [thyroid stimulating hormone] levels in 2, but without clinical features of hypothyroidism. Seven of the patients in this study were treated with prednisone and cyclophosphamide. Three achieved complete remission but only 1 patient has survived longer than 2 yr. Failure to achieve complete remission was associated with a 90% mortality within 1 yr of the onset of disease in the reports reviewed. Corticosteroids alone have produced only a 40% rate of remission. The management of poor responders, non-responders and many relapse cases was unsatisfactory. More intensive chemotherapy is very hazardous, increasing the already high risk of severe infections, and is often unsuccessful. Histological features recently reported to represent co-existent immunoblastic lymphoma at diagnosis were recognized in 3 patients, 2 of which went on to develop definite lymphoma.

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