Cabrera M E, Labra S, Catovsky D, Ford A M, Colman S M, Greaves M F, Matutes E
Department of Medicine, Faculty of Medicine, University of Chile, Hospital del Salvador, Santiago de Chile.
Leukemia. 1994 Oct;8(10):1763-7.
We describe the clinical and laboratory features of nine patients born in Chile with HTLV-I-positive adult T-cell leukemia/lymphoma (ATLL). All were adults (median age 51 years) of Caucasian origin without evidence of Indian or foreign extraction and none had been out of the country. The main disease features were organomegaly, cutaneous lesions, hypercalcemia and leukemia with atypical polylobed lymphocytes displaying a CD2+, CD3+, CD4+, CD8-, CD7- T-cell phenotype. Eight patients presented with acute type ATLL and one had a chronic form lasting for 16 months prior to the development of the acute phase. Lymph node histology (three cases) was consistent with a T-cell non-Hodgkin's lymphoma (large and small cells). Antibodies to HTLV-I were detected by ELISA and particle agglutination in the serum from eight of nine patients. DNA analysis showed HTLV-I proviral DNA in all seven cases investigated, including the single serologically negative patient. In five cases, HTLV-I was monoclonally integrated and in one case oligoclonal. In the seventh case viral DNA clonal status was ambiguous. Response to therapy was poor and median survival was 3 months (range 2-20 months). This study provides further evidence that HTLV-I is endemic in Chile, a non-tropical country where the two main diseases associated with HTLV-I, ATLL and TSP, are found.
我们描述了9名在智利出生的成人T细胞白血病/淋巴瘤（ATLL）患者的临床和实验室特征。所有患者均为白种人成年人（中位年龄51岁），无印第安或外国血统证据，且均未出过国。主要疾病特征包括器官肿大、皮肤病变、高钙血症以及白血病，伴有显示CD2 +、CD3 +、CD4 +、CD8 -、CD7 - T细胞表型的非典型多叶淋巴细胞。8例患者表现为急性型ATLL，1例为慢性型，在急性期发展前持续了16个月。淋巴结组织学检查（3例）与T细胞非霍奇金淋巴瘤（大细胞和小细胞）一致。9例患者中有8例血清通过ELISA和颗粒凝集法检测到抗HTLV - I抗体。DNA分析显示，在所研究的所有7例病例中均存在HTLV - I前病毒DNA，包括1例血清学阴性患者。5例中HTLV - I呈单克隆整合，1例为寡克隆。第7例病毒DNA克隆状态不明确。治疗反应较差，中位生存期为3个月（范围2 - 20个月）。本研究进一步证明，HTLV - I在智利呈地方性流行，智利是一个非热带国家，却发现了与HTLV - I相关的两种主要疾病，即ATLL和热带痉挛性截瘫（TSP）。
