T-cell malignancies in Brazil. Clinico-pathological and molecular studies of HTLV-I-positive and -negative cases

✍ Scribed by Maria S. Pombo De Oliveira; Estella Matutes; Thomas Schulz; Silvia M. Carvalho; Helio Noronha; Jacqueline D. Reaves; Paula Loureiro; Cintia Machado; Daniel Catovsky

• Book ID: 102870021
• Publisher: John Wiley and Sons
• Year: 1995
• Tongue: French
• Weight: 517 KB
• Volume: 60
• Category: Article
• ISSN: 0020-7136
• DOI: 10.1002/ijc.2910600617

✦ Synopsis

Abstract

T‐cell malignancies in Brazil have a high seroprevalence rate of HTLV‐I antibodies. We have analyzed the disease features in 188 Brazilian patients with a T‐cell disorder. These included 40 with T‐lymphoblastic leukaemia or lymphoma (T‐ALL/T‐LbLy) and 148 with mature T‐cell diseases: 5 T‐prolymphocytic leukaemia, 53 adult T‐cell leukaemia/lymphoma (ATLL), 54 cutaneous T‐cell lymphomas, 29 pleomorphic T‐cell lymphomas and 7 large granular lymphocyte leukaemia. The diagnosis was based on clinical, morphological and immunological features and HTLV‐I serology. ATLL in Brazil has the same disease features as in other endemic regions, the only apparent differences being: age, Brazilian patients being younger than Japanese, and ethnic grouping, one third of Brazilians being white Caucasians of European descent. We applied a scoring system based on the presence or absence of typical features associated with ATLL: hypercalcaemia, cell morphology, immunophenotype, histopathology and HTLV‐I status, to see whether it may help in diagnosing cases of ATLL. All had high scores, whereas all other T‐cell diseases scored low. Only 5 ATLL cases were HTLV‐Inegative by serology, but they had otherwise typical features of ATLL, and their cells did not have HTLV‐I proviral sequences by DNA analysis. Such cases suggest that ATLL may develop in a minority of individuals living in regions where it is endemic, without evidence of HTLV‐I infection, and that other factors may contribute to the pathogenesis of the disease.