Adult T-cell leukemia/lymphoma in London: clinical experience of 21 cases

Pawson, R.; Richardson, D.S.; Pagliuca, A.; Kelsey, S.M.; Hoque, S.; Breuer, J.; Newland, A.C.; Mufti, G.J.

Leukemia and Lymphoma 31(1-2): 177-185

1998


ISSN/ISBN: 1042-8194
PMID: 9720727
DOI: 10.3109/10428199809057597
Accession: 045180312

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Abstract
Adult T-cell leukemia/lymphoma (ATLL) is uncommon in the United Kingdom and has so far been restricted to people of Afro-Caribbean extraction. Between 1981 and 1995, 21 cases presented to 2 inner London teaching hospitals where 17% of the population are of Afro-Caribbean origin. Clinical presentations were similar to those of the disease in HTLV-I-endemic areas. Major responses (CR + PR) were obtained in 10/16 assessable patients (63%) treated with combination chemotherapy. However, median survival was only 5.5 months. Disease progression and opportunistic infection were the major causes of treatment failure and death. Three patients (14%) relapsed in the central nervous system (CNS). Our cases confirm the profound immunosuppression in ATLL. The poor prognosis of acute and lymphoma types of ATLL highlight the need for new approaches to treatment such as zidovudine and alpha-interferon, incorporating prophylaxis against CNS disease and opportunistic infections.