Geographic diversity of adult T-cell leukemia/lymphoma in Brazil

We describe 195 cases of adult T-cell leukemia/lymphoma (ATLL) reported to the national registry of T-cell malignancies in Brazil between 1994 and 199 8. We compared the effect of demographic differences and clinical features of 150 consecutive ATLL cases in different regions of this diverse country. At diagnosis, the predominant clinical sub-type was the acute type (60%), followed by lymphoma (22%)(v) chronic (10%) and smoldering (8%) types. Alth ough we expected that different sub-types would be present in different reg ions, on the basis of immunogenetic factors determined by ethnicity, we did not demonstrate these differences. There were no significant differences a mong ATLL subtypes by age or gender. No ethnic group predominated in the to tal population of patients, but significant differences were noted when exa mining ethnic distribution by region. Reflecting the general population dis tribution, white patients were seen more often in Sao Paulo and black patie nts in Bahia, than in other regions, In most regions, cases were equally di stributed between blacks and mulattos, except in Pernambuco, where blacks w ere less frequent. The main clinical features were lymphadenopathy, skin le sions, hypercalcemia and hepatomegaly. Fourteen patients (9%) suffered from HTLV-I-associated myelopathy (HAM/TSP), either at diagnosis or during foll ow-up of ATLL. All cases but one had antibodies to HTLV-I, with concordant results with ELISA, WE and FCR analyses. For the antibody-negative case, po l and tax gene sequences were present in tumor cells when subjected to PCR analyses. The prognosis was generally poor, suggesting that the disease in Brazil behaves in similar fashion regardless of ethnic or geographical diff erences. (C) 1999 Wiley-Liss, Inc.