Angio-immunoblastic lymphadenopathy: A clinical, immunological and molecular study

• Published in: British journal of cancer Vol. 55; no. 4; pp. 437 - 442
• Main Authors: GANESAN, T. S, DHALIWAL, H. S, DORREEN, M. S, STANSFELD, A. G, HABESHAW, J. A, LISTER, T. A
• Format: Journal Article
• Language: English
• Published: Basingstoke Nature Publishing Group 01.04.1987
• Subjects: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Biological and medical sciences; Child; Cyclophosphamide - therapeutic use; Female; Hematologic and hematopoietic diseases; Humans; Immunoblastic Lymphadenopathy - drug therapy; Immunoblastic Lymphadenopathy - immunology; Immunoblastic Lymphadenopathy - pathology; Immunoglobulins - genetics; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Leukocyte Count; Lymph Nodes - pathology; Male; Mechlorethamine - therapeutic use; Medical sciences; Middle Aged; Phenotype; Prednisolone - therapeutic use; Procarbazine - therapeutic use; Receptors, Antigen, T-Cell - genetics; T-Lymphocytes, Helper-Inducer; T-Lymphocytes, Regulatory; Vinblastine - therapeutic use; Human; Immunohistochemistry; Immunopathology; Nosology; Hemopathy; Non Hodgkin lymphoma; Peripheral T cell lymphoma; Beta-»Peptide chain; Immunogenetics; Phenotype; Membrane receptor; Gene; Immunological investigation; Polyclonal immunoglobulinemia; Immunoblastic lymphadenopathy; T-Lymphocyte
• ISSN: 0007-0920; 1532-1827
• DOI: 10.1038/bjc.1987.86

Twenty four patients with angio-immunoblastic lymphadenopathy (AILD) presenting between 1974 and 1985 have been reviewed. Clinical features at presentation included rash, fever, lymphadenopathy and hepatosplenomegaly in 75% of patients. Polyclonal hypergammaglobulinaemia was seen in 19/20 patients; 5 had normal immunoglobulin levels. Combination chemotherapy with MVPP was the optimal treatment with 6/7 patients achieving complete remission. Duration of remission ranged from 9 months to 4 years and was significantly longer in patients achieving complete as opposed to partial remission. In 6 patients phenotype studies were performed on single cell suspensions and immunoperoxidase studies on frozen sections of 7 lymph nodes. There was a reversal of the normal T suppressor/helper cell ratio with a predominance of T suppressor cells. Loss of normal B follicles was observed histologically in all except one lymph node. Germline configuration of the beta B-chain of the T cell receptor was observed in lymph nodes of 4 patients with AILD, and a rearranged T cell receptor was observed in 1 patient in whom a second lymph node biopsy had shown alteration of the histological picture to that of T-zone lymphoma. Frozen sera of 15 patients were screened for antibodies to HTLV I and III and were found to be negative.