title: T-cell malignancies in Brazil. Clinico-pathological and molecular studies of HTLV-I-positive and -negative cases.
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 diseases 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-I-negative 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.
authors.affiliation: Cell Markers Laboratory of Cancer Hospital, Rio de Janeiro, Brazil.
authors.forename: M S
authors.initials: MS
authors.lastname: Pombo de Oliveira
authors.forename: E
authors.initials: E
authors.lastname: Matutes
authors.forename: T
authors.initials: T
authors.lastname: Schulz
authors.forename: S M
authors.initials: SM
authors.lastname: Carvalho
authors.forename: H
authors.initials: H
authors.lastname: Noronha
authors.forename: J D
authors.initials: JD
authors.lastname: Reaves
authors.forename: P
authors.initials: P
authors.lastname: Loureiro
authors.forename: C
authors.initials: C
authors.lastname: Machado
authors.forename: D
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001483:Base Sequence; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D015368:Human T-lymphotropic virus 1 / Q000302:isolation & purification*; D006801:Humans; D007223:Infant; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology / Q000821:virology; D016410:Lymphoma, T-Cell, Cutaneous; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D016036:Seroepidemiologic Studies