de Mendoza Carmen, Rando Ariadna, Miró Elisenda, Pena María José, Rodríguez-Avial Iciar, Ortega Diego, González-Praetorius Alejandro, Reina Gabriel, Pintos Ilduara, Pozuelo María José, Soriano Vicente
Puerta de Hierro University Hospital & Research Foundation-IDIPHISA, Madrid, Spain.
Vall d'Hebrón University Hospital, Barcelona, Spain.
J Clin Virol. 2023 Oct;167:105578. doi: 10.1016/j.jcv.2023.105578. Epub 2023 Aug 28.
HTLV-1 infection is a neglected disease, despite producing neurological and lymphoproliferative severe illnesses and affect over 10 million people worldwide. Roughly 5% of HTLV-1 carriers develop Adult T-cell leukemia/lymphoma (ATLL), one of the most aggressive hematological malignancies.
A national HTLV-1 register exists since 1989 in Spain, a non-endemic country with a large migrant flow from Latin America and Equatorial Africa, where HTLV-1 is endemic. The main features of all patients diagnosed with ATLL in Spain up to date are reported.
A total of 451 cases of HTLV-1 infection had been reported in Spain until the end of year 2022. ATLL had been diagnosed in 35 (7.8%). The current average incidence of ATLL in Spain is of two cases per year. Women represent 57% of ATLL patients. Mean age at diagnosis was 47 years-old. Roughly 57% were Latin Americans and 26% Africans. At diagnosis, the majority presented with acute or lymphoma clinical forms. Survival was shorter than one year in most of them. Mean HTLV-1 proviral load was significantly greater in ATLL patients than in asymptomatic HTLV-1 carriers (2,305 vs 104 copies/10 PBMC). HTLV-1 subtyping in 6 ATLL patients found the 1a transcontinental variant (n = 4) and the Japanese variant (n = 2). All ATLL patients were negative for HIV-1, did not develop HTLV-1-associated myelopathy and were not transplant recipients.
The rate of ATLL is very low in Spain and mostly associated to migrants from HTLV-1 endemic regions. Given the poor clinical outcome of ATLL, HTLV-1 testing should be performed at least once in all migrants coming from HTLV-1 endemic countries and in natives who have lived in or had sex partners from such regions.
尽管人类嗜T淋巴细胞病毒1型（HTLV-1）感染会引发神经系统和淋巴增殖性严重疾病，且全球有超过1000万人受其影响，但它仍是一种被忽视的疾病。大约5%的HTLV-1携带者会发展为成人T细胞白血病/淋巴瘤（ATLL），这是最具侵袭性的血液系统恶性肿瘤之一。
自1989年起，西班牙建立了全国性的HTLV-1登记册，该国并非HTLV-1流行国家，但有大量来自拉丁美洲和赤道非洲的移民，而HTLV-1在这些地区呈地方性流行。本文报告了西班牙截至目前所有被诊断为ATLL的患者的主要特征。
截至2022年底，西班牙共报告了451例HTLV-1感染病例。其中35例（7.8%）被诊断为ATLL。西班牙目前ATLL的年平均发病率为每年2例。女性占ATLL患者的57%。诊断时的平均年龄为47岁。大约57%为拉丁美洲人，26%为非洲人。诊断时，大多数患者表现为急性或淋巴瘤临床形式。他们中的大多数人存活时间短于一年。ATLL患者的平均HTLV-1前病毒载量显著高于无症状HTLV-1携带者（2305对104拷贝/10个外周血单核细胞）。对6例ATLL患者进行的HTLV-1亚型分析发现了1a跨大陆变体（n = 4）和日本变体（n = 2）。所有ATLL患者的HIV-1检测均为阴性，未发展为HTLV-1相关脊髓病，也不是移植受者。
西班牙的ATLL发病率非常低，且大多与来自HTLV-1流行地区的移民有关。鉴于ATLL的临床预后较差，对于所有来自HTLV-1流行国家的移民以及曾在这些地区居住或有性伴侣的本国居民，应至少进行一次HTLV-1检测。
