Department of Medicine, Division of Hematology and Medical Oncology, State University of New York (SUNY) Downstate Medical Center, Brooklyn, NY.
Department of Pathology, SUNY Downstate Medical Center, Brooklyn, NY.
JCO Glob Oncol. 2020 Apr;6:548-556. doi: 10.1200/JGO.19.00208.
Adult T-cell lymphoma/leukemia ATL) is a rare and aggressive peripheral T-cell malignancy caused by human T-cell lymphotropic virus-1 infection, which occurs in areas of high prevalence, predominantly in Japan and the Caribbean basin. Most ATL literature is derived from Japan and little is published about Caribbean patients. We describe the clinicopathologic characteristics and treatment outcomes of our Caribbean patients who have ATL at the State University of New York Downstate Medical Center and Kings County Hospital.
We conducted a retrospective analysis of our patients with ATL who were diagnosed between 2005 and 2017. Medical records were reviewed for clinicopathologic data and treatment outcomes. The final analysis included acute and lymphomatous subtypes only. For the univariable analysis, outcomes were calculated by using a log-rank test, and survival curves were estimated by the Kaplan-Meier method.
We identified 63 patients with acute (55%) and lymphomatous (45%) subtypes, 95% of whom had Ann Arbor stage III to IV disease. The median age was 54 years, and the study population was predominantly female (65%). Most patients (82%) received first-line etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone (EPOCH) or cyclophosphamide, vincristine, doxorubicin, and prednisone (CHOP) chemotherapy (10%) with an overall response rate of 46%. The median overall survival was 5.5 months, and the median progression-free survival was 4 months. Incidence of atypical immunophenotype (32%) was higher than previously reported in the Japanese literature and was associated with worse survival ( = .04). Abnormal cytogenetics correlated with shorter progression-free survival ( < .05).
We describe here the clinicopathologic characteristics and treatment outcomes of our Caribbean patients with aggressive ATL, which is largely chemotherapy resistant, and the challenges of treating a population with unmet medical needs.
成人 T 细胞淋巴瘤/白血病(ATL)是一种罕见且侵袭性的外周 T 细胞恶性肿瘤,由人类 T 细胞嗜淋巴细胞病毒 1 感染引起,主要发生在高流行地区,主要在日本和加勒比海地区。大多数 ATL 文献来源于日本,关于加勒比地区患者的报道很少。我们描述了在纽约州立大学下州医学中心和国王郡医院就诊的 ATL 加勒比患者的临床病理特征和治疗结果。
我们对 2005 年至 2017 年间诊断为 ATL 的患者进行了回顾性分析。对病历进行了临床病理数据和治疗结果的回顾性分析。最终分析仅包括急性和淋巴瘤亚型。对于单变量分析,使用对数秩检验计算结果,通过 Kaplan-Meier 方法估计生存曲线。
我们确定了 63 例急性(55%)和淋巴瘤(45%)亚型患者,其中 95%的患者患有安纳堡分期 III 至 IV 期疾病。中位年龄为 54 岁,研究人群以女性为主(65%)。大多数患者(82%)接受一线依托泊苷、环磷酰胺、长春新碱、多柔比星和泼尼松(EPOCH)或环磷酰胺、长春新碱、多柔比星和泼尼松(CHOP)化疗(10%),总缓解率为 46%。中位总生存期为 5.5 个月,中位无进展生存期为 4 个月。非典型免疫表型(32%)的发生率高于日本文献中的报道,且与生存时间更差相关( <.05)。异常细胞遗传学与无进展生存期较短相关( <.05)。
我们在这里描述了我们的加勒比地区侵袭性 ATL 患者的临床病理特征和治疗结果,这些患者的化疗耐药性很大,并且在治疗有未满足医疗需求的人群方面存在挑战。