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Adult T-cell leukemia/lymphoma in Jujuy,north-west Argentina
Authors:Marin Oscar  Hasui Kazuhisa  Remondegui Carlos  Sato Eiichi  Aye Moe Moe  Takenouchi Norihiro  Izumo Shuji  Tajima Kazuo
Institution:Department of Pathology, Pablo Soria Hospital, Argentina.
Abstract:Human T-cell leukemia virus type 1 (HTLV-1) infection is prevalent in native Americans living in the Andes. Some of their malignant lymphomas (ML) show a peculiar histology suggestive of adult T-cell leukemia/lymphoma (ATLL). To determine whether ML resembling ATLL are indeed ATLL, re-analysis of 34 cases occurring in Jujuy, a province of Argentina, was conducted, concentrating on immunological phenotype, integration of HTLV-1 proviral DNA, expression of HTLV-1 p40Tax and p27Rex, and infection of Epstein-Barr virus (EBV). The ML were 22 cases of mature peripheral T-cell and natural killer (NK)-cell neoplasm (mT/NKN), 11 B-cell malignant neoplasms and one Hodgkin's lymphoma. Polymerase chain reaction against the HTLV-1 proviral DNA, using DNA extracted from paraffin sections, indicated integration of the HTLV-1 proviral DNA in three cases of eight mT/NKN. Two other cases of mT/NKN were positive for anti-HTLV-1 antibodies. Expression of p40Tax and p27Rex was detected in all five of these mT/NKN cases associated with HTLV-1. As such, these five mT/NKN were rediagnosed as ATLL. In situ hybridization signals for EBV-encoded small nuclear early region-1 were detected in nine cases of mT/NKN, of which five cases of NK-cell lymphoma were found to have cytoplasmic CD3 expression, a CD56 phenotype and positivity of TIA1. According to the new World Health Organization classification, the mT/NKN class includes five cases of ATLL and five cases of NK-cell lymphomas. The five cases of ATLL were of native American extraction from an HTLV-1-endemic area around Jujuy, north-west Argentina.
Keywords:adult T-cell leukemia  lymphoma (ATLL)  Argentina  Epstein–Barr virus (EBV)  HTLV-1 proviral DNA  human T-cell leukemia virus type 1 (HTLV-1)  modified ImmunoMax  native American  NK-cell lymphoma  p40Tax and p27Rex  paraffin-immunohistochemistry  pathoepidemiology  polymerase chain reaction  World Health Organization classification
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