The purpose of this study was to evaluate whether subdivision of adult T-cell leukemia/lymphoma (ATL) on the basis of clinical types, skin involvement, histologic features, cell size, and proliferative index (PI) was clinically relevant. Skin lesions were present in 47 cases (67%). Five cases were classified as primary cutaneous tumoral (PCT) type not included in the Shimoyama classification and characterized by skin tumors and absence of systemic involvement, lymphocytosis, and hypercalcemia. Mortality was high (61/70 [87%]). The overall median survival time (MST) was 12 months. The following variables were adversely related to survival: acute, lymphoma, and PCT types; absence of skin lesions; large cells; and PI more than 18%. The longer MST observed in cases with skin lesions was probably due to prolonged survival of the smoldering type (58 months). The MST of the PCT type (21 months) was shorter than that of the smoldering type, confirming the importance of clearly defining these 2 types of ATL.
Pediatric Hodgkin lymphoma (HL) occurring in developing regions is different from HL in industrialized countries due to the higher frequency of association with Epstein-Barr virus (EBV) infection. This infection is related to classical HL (cHL) but is virtually absent in nodular lymphocyte predominant HL (nLPHL). We studied the phenotype and the expression of EBV gene products in 90 pediatric cases by immunohistochemistry and in situ hybridization. EBV-positive tumor cells were found exclusively in cHL. The infection occurred with high frequency in all cHL subtypes, but it predominated in the mixed cellularity and lymphocyte depletion subtypes. These results reinforce the hypothesis that EBV plays a major role in the etiology of pediatric cHL in developing areas. Curiously, the frequency of EBV infection in HL was identical to the previously described for Burkitt's lymphoma in the same pediatric population. As both lymphomas have a postulated precursor cell in the germinal center (GC), the pattern of latently EBV-infected GC cells previously described in Bahia may be related to the development of these lymphomas.
The state of Bahia in the northeastern coast of Brazil is a region in which HTLV-I infection is endemic. This study investigated the characteristics of 28 HTLV-I-associated lymphomas/leukemias in this region. HTLV-I-infection diagnosis was based on serologic study, Southern blot analysis, and polymerase chain reaction (PCR) in neoplastic tissue. The main clinical differences between these lymphomas and adult T-cell leukemia (ATL) cases from other endemic areas were as follows. The mean age was 47 years; 20% of the cases occurred in young adults; and a predominance was found among male subjects (2:1), blacks, and of those of mixed race (96%). Histologically, 20 cases were T-cell pleomorphic leukemia/lymphoma, 5 were Mycosis fungoides-like cutaneous lymphoma, and 3 were CD30+ large-cell anaplastic lymphoma. Immunohistochemistry demonstrated 4 cases of CD8+ lymphoma. Proviral genomic sequences were demonstrated by PCR in 9 lymph node biopsy specimens and in 3 skin biopsy specimens. Southern blot was performed and was positive in 8 cases.
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