Angio-immunoblastic lymphadenopathy: report of ten cases and review of the literature.

Abstract

Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknown aetiology and pathogenesis. It has features of hyperimmunity, immune deficiency and can behave like a malignant lymphoma. An underlying abnormality of T-cell regulatory function has been proposed. We report ten patients with AIL followed prospectively and review 200 cases from the literature. As well as showing the typical features described in previous retrospective series namely: constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin eruptions, hypergammaglobulinaemia and characteristic lymph node histology; four patients had oedema with ascites or pleural effusions associated with hyponatraemia and hypoalbuminaemia. We have also observed low free thyroxine indices in three patients with elevated TSH levels in two, 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 one patient has survived longer than two years. Failure to achieve complete remission has been associated with a 90 per cent mortality within one year of the onset of disease in the reports reviewed. Corticosteroids alone have produced only a 40 per cent rate of remission. The management of poor responders, non-responders and many relapse cases in unsatisfactory. More instensive 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 three of our patients, two of which went on to develop definite lymphoma.