Real-World Data on Adult T-Cell Leukemia/Lymphoma in Latin America: A Study From the Grupo de Estudio Latinoamericano de Linfoproliferativos.
Real-World Data on Adult T-Cell Leukemia/Lymphoma in Latin America: A Study From the Grupo de Estudio Latinoamericano de Linfoproliferativos.
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DOI：
10.1200/go.21.00084
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发表时间：
2021-07
影响因子：
4.5
通讯作者：
Beltran BE
中科院分区：
文献类型：
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作者：
Malpica L;Enriquez DJ;Castro DA;Peña C;Idrobo H;Fiad L;Prates M;Otero V;Biglione M;Altamirano M;Sandival-Ampuero G;Aviles-Perez U;Meza K;Aguirre-Martinez L;Cristaldo N;Maradei JL;Guanchiale L;Soto P;Viñuela JL;Cabrera ME;Paredes SR;Riva E;Di Stefano M;Noboa A;Choque JA;Candelaria M;Von Glasenapp A;Valvert F;Torres-Viera MA;Castillo JJ;Ramos JC;Villela L;Beltran BE
成人T细胞白血病/淋巴瘤（ATLL）是由人类T细胞白血病病毒1型引起的侵袭性疾病。缺乏拉丁美洲ATLL的真实世界数据。我们分析了1995年至2019年期间在11个拉丁美洲国家遇到的ATLL（急性，淋巴瘤，慢性和阴燃）患者。根据2009年共识报告评估治疗反应。使用Kaplan-Meier方法和对数秩检验估计存活曲线。我们确定了253例患者; 226例（淋巴瘤：n = 122，急性：n = 73，慢性：n = 26，阴燃：n = 5）有足够的数据进行分析（中位年龄57岁）。大多数ATLL患者来自秘鲁（63%）、智利（17%）、阿根廷（8%）和哥伦比亚（7%）。高钙血症与急性型呈正相关（57%对淋巴瘤型27%，P = 0.014）。中位生存时间（月）分别为4.3、7.9、21.1，急性、淋巴瘤、慢性和阴燃型未达到，4年生存率分别为8%、22%、40%和80%。一线齐多夫定（AZT）-干扰素α（IFN）导致急性的总反应率为63%（完全反应[CR] 24%）。一线化疗对淋巴瘤的总有效率为41%（CR 29%）。足叶乙甙、环磷酰胺、长春新碱、阿霉素和泼尼松的CR率为42%，环磷酰胺、长春新碱、阿霉素和泼尼松类方案的CR率为12%（P <0.001）。AZT-IFN治疗的急性型患者1年无进展生存率为67%，而化疗后达到CR的淋巴瘤型患者2年无进展生存率为77%。本研究证实，与日本患者相比，拉丁美洲ATLL的发病年龄更小，淋巴瘤型的发病率更高，惰性亚型的发病率更低，生存率更低。在侵袭性ATLL中，化疗仍然是淋巴瘤的首选，支持依托泊苷为基础的方案（依托泊苷，环磷酰胺，长春新碱，阿霉素和泼尼松），而AZT-IFN仍然是急性亚型的良好一线选择。
Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone–like regimen (P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous type patients who achieved CR after chemotherapy was 77%. This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.