Universidad Peruana Cayetano Heredia

Epidemiology, clinical features, and outcome of HTLV-1–related ATLL in an area of prevalence in the United States

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dc.contributor.author Malpica, Luis
dc.contributor.author Pimentel, Agustin
dc.contributor.author Reis, Isildinha M.
dc.contributor.author Gotuzzo Herencia, José Eduardo
dc.contributor.author Lekakis, Lazaros
dc.contributor.author Komanduri, Krishna
dc.contributor.author Harrington, Thomas
dc.contributor.author Barber, Glen N.
dc.contributor.author Ramos, Juan C.
dc.date.accessioned 2018-08-10T20:11:15Z
dc.date.available 2018-08-10T20:11:15Z
dc.date.issued 2018
dc.identifier.uri https://hdl.handle.net/20.500.12866/3770
dc.description.abstract Adult T-cell leukemia/lymphoma (ATLL) is a fatal disease caused by human T-cell leukemia virus type 1 (HTLV-1). We retrospectively analyzed 195 patients with ATLL (lymphomatous n = 96, acute n = 80, unfavorable chronic n = 7, chronic n = 5, smoldering n = 3, and unclassified n = 4) diagnosed between 1987 and 2016 (median age 52 years, 77% Afro-Caribbean). Hypercalcemia was associated with acute ATLL (65%, vs 23% lymphomatous) (P = .012). The median survival for patients treated with modern therapies between 2000 and 2016 was 4.1 months for acute, 10.2 months for lymphomatous, 72 months for chronic/smoldering, and not reached for unfavorable chronic type, with 4-year survival rates of 10%, 4%, 60%, and 83%, respectively. The overall response rate (ORR) after first-line multiagent chemotherapy was 78% (complete response [CR] 39%) for acute vs 67% (CR 33%) for lymphomatous ATLL. First-line zidovudine interferon-α (AZT-IFN) resulted in ORR of 56% (CR 23%) for acute (n = 43), 33% (CR 16.5%) for lymphomatous (n = 6), and 86% (CR 29%) for unfavorable chronic ATLL. The median progression-free survival (PFS) in patients with aggressive ATLL who achieved CR after AZT-IFN was 48 months vs 11 months after chemotherapy (P = .003). Allogeneic hematopoietic stem cell transplant (allo-HSCT) resulted in a PFS of 24 and 28 months in 2 patients with lymphomatous ATLL. Our results suggest high-dose AZT-IFN is a reasonable up-front option for patients with aggressive leukemic ATLL followed by chemotherapy switch in nonresponders, whereas chemotherapy should be used in lymphomatous type followed by allo-HSCT when feasible. en_US
dc.language.iso eng
dc.publisher Elsevier
dc.relation.ispartofseries Blood advances
dc.rights info:eu-repo/semantics/restrictedAccess
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es
dc.subject Clinical Trials and Observations en_US
dc.subject Lymphoid Neoplasia en_US
dc.title Epidemiology, clinical features, and outcome of HTLV-1–related ATLL in an area of prevalence in the United States en_US
dc.type info:eu-repo/semantics/article
dc.identifier.doi https://doi.org/10.1182/bloodadvances.2017011106
dc.subject.ocde https://purl.org/pe-repo/ocde/ford#3.02.06
dc.relation.issn 2473-9537


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