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 1998. 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. Although we expected that different sub-types would be present in different regions, on the basis of immunogenetic factors determined by ethnicity, we did not demonstrate these differences. There were no significant differences among ATLL subtypes by age or gender. No ethnic group predominated in the total population of patients, but significant differences were noted when examining ethnic distribution by region. Reflecting the general population distribution, white patients were seen more often in Sao Paulo and black patients in Bahia, than in other regions, In most regions, cases were equally distributed between blacks and mulattos, except in Pernambuco, where blacks were less frequent. The main clinical features were lymphadenopathy, skin lesions, hypercalcemia and hepatomegaly. Fourteen patients (9%) suffered from HTLV-I-associated myelopathy (HAM/TSP), either at diagnosis or during follow-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, pol 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 differences. (C) 1999 Wiley-Liss, Inc.Canc Hosp, Inst Nacl Canc, Cell Markers Lab, Rio De Janeiro, BrazilHEMOPE, Dept Hematol, Recife, PE, BrazilSch Med, Dept Pathol, Salvador, BA, BrazilUniv Fed Sao Paulo, Dept Hematol, Sao Paulo, BrazilMed Sch Santa Casa da Misercordia, Sao Paulo, BrazilInst Estadual Hematol, Immunol Lab, Rio De Janeiro, BrazilNew York Blood Ctr, New York, NY 10021 USAUniv Maryland, Inst Human Virol, Baltimore, MD 21201 USAUniv Fed Sao Paulo, Dept Hematol, Sao Paulo, BrazilWeb of Scienc