THE EFFECT OF ETHNIC-DIFFERENCES ON THE PATTERN OF HTLV-I-ASSOCIATED T-CELL LEUKEMIA-LYMPHOMA (HATL) IN THE UNITED-STATES

Human T-cell lymphotropic virus Type I (HTLV-I) is the primary etiolog ic factor for adult T-cell leukemia/lymphoma (ATL). Although HTLV-I is endemic in Japan and the Caribbean islands, the reported clinical and epidemiologic features of ATL in these 2 parts of the world are quite different. ATL has been diagnosed at a younger age and is reported mo re frequently as the lymphomatous type rather than the acute type with leukemia in the Caribbean basin as compared with the presentation in Japan. In order to characterize ATL in the United States, a registry h as been established at the National Cancer Institute for the purpose o f recording all cases originally diagnosed in the United States. This registry was utilized to examine the effect of ethnic differences on a ge of onset and clinical features of ATL, using the same data base. Cl inical and laboratory information was obtained from 177 patients suspe cted of having ATL, who were treated at the National Institutes of Hea lth, or had biological samples sent for evaluation, or were reported i n the literature. Histopathologic review and virologic studies were pe rformed by standardized methods. Of 177 patients registered, 127 were considered as having ATL, according to an algorithm combining clinical , pathologic and laboratory features. Presenting features in the confi rmed cases consisted primarily of lymphadenopathy (76.6%), hypercalcem ia (72.5%), leukemia (82%), skin involvement (48.2%) and hepatomegaly (53.6%). Patients of Japanese ancestry were generally older (median ag e 63, range 51 to 73 years) than patients of African-American descent (median age 39, range 7 to 75 years) and presented more often with leu kemia (90 vs. 69%). Of the 103 cases where country of birth was confir med, 45 (43.7%) were born in the United States. The prognosis was gene rally poor (median survival 3.24 months), but rare long-term remission s were documented. (C) 1994 Wiley-Liss, Inc.