Adult T-cell leukemia/lymphoma in HTLV-1 non-endemic regions

de Mendoza, Carmen ; Rando, Ariadna ; Miró, Elisenda ; Pena, María José ; Rodríguez-Avial, Iciar ; Ortega, Diego (Universidad de Zaragoza) ; González-Praetorius, Alejandro ; Reina, Gabriel ; Pintos, Ilduara ; Pozuelo, María José ; Soriano, Vicente ; Calderón, E. ; Rodríguez-Iglesias, M. ; Montiel, N. ; Trujillo, T. ; Viciana, I. ; Cabezas, T. ; Lozano, A. ; Fernández-Fuertes, E. ; Fernández, J.M. ; García, F. ; Benito, R. (Universidad de Zaragoza) ; Algarate, S. (Universidad de Zaragoza) ; Ducons, M. ; Roc, L. ; Cifuentes, C. ; Fernández-Baca, V. ; Fernández-Montero, J.V ; Maciá, MD. ; Hernández-Betancor, A. ; Martín, A.M. ; Hernández, M. ; López-Lirola, A.M. ; Gómez-Sirvent, J.L. ; Copado, R. ; Cano, M.E. ; Rojo, S. ; Eirós, J.M. ; Rodríguez, M. ; Gómez-Hernando, C. ; Force, L. ; Cebollero, A. ; Delgado, J.F. ; Rodríguez, G. ; Fernández-Pereira, L. ; Aguilera, A. ; Pereira, S. ; García, J. ; Trigo, M. ; Diz, J. ; García-Campello, M. ; Cortizo, S. ; Pérez, S. ; Morano, L. ; Arazamendi, M. ; Salicio, Y. ; Ugalde, E. ; Nieto, M.C. ; Liendo, P. ; Goikoetxea, A.J. ; Ocete, M.D. ; Ramos, J.M. ; Escribano, I. ; Sauleda, S. ; Pirón, M. ; González, R. ; Richart, A. ; Barea, L. ; Jiménez, A. ; Blanco, L. ; Navarro, L. ; Ayerdi, O. ; Baza, B. ; Rodriguez, C. ; del Romero, J. ; Galar, A. ; Aldamiz, T. ; Valeiro, M. ; Pérez, L. ; Martín-Carbonero, L. ; Fernández-Ruiz, M. ; Parra, P. ; Redondo, N. ; Ruiz-Merlo, T. ; Barreiro, P. ; Treviño, A. ; Corral, O. ; Moreno-Torres, V. ; Carrizo, P. ; Huertas, A. ; Vargas-Núñez, J.A.

Resumen: Background
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.
Methods
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.
Results
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/104 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.
Conclusion
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.

Idioma: Inglés

DOI: 10.1016/j.jcv.2023.105578

Año: 2023

Publicado en: JOURNAL OF CLINICAL VIROLOGY 167 (2023), 105578 [5 pp.]
ISSN: 1386-6532

Financiación: info:eu-repo/grantAgreement/ES/ISCIII/FIS/PI21-1717

Tipo y forma: Artículo (Versión definitiva)

Área (Departamento): Área Microbiología (Dpto. Microb.Ped.Radio.Sal.Pú.)