Clinical Features and Treatment Outcomes of 81 Patients with Aggressive Type Adult T-cell Leukemia-lymphoma at a Single Institution over a 7-year Period (2006-2012)
Clinical Features and Treatment Outcomes of 81 Patients with Aggressive Type Adult T-cell Leukemia-lymphoma at a Single Institution over a 7-year Period (2006-2012)
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DOI：
10.2169/internalmedicine.54.1953
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发表时间：
2015-01-01
影响因子：
1.2
通讯作者：
Ueda, Akira
中科院分区：
文献类型：
--
作者：
Kawano, Noriaki;Yoshida, Shuro;Ueda, Akira
目的尽管化疗和异基因造血干细胞移植（HSCT）取得了显著进展，但成人T细胞白血病淋巴瘤（ATL）仍然是一个高死亡率的疾病。因此，有必要阐明目前的特点ATL.Methods我们回顾性分析了81例患者在我们的机构超过7年的Shimoyama的诊断critics.Results 81例中位年龄为67.5岁的基础上，积极型ATL被归类为急性（n=47），淋巴瘤（n=32），或慢性型（n=2）ATL。他们最初接受姑息治疗（n=25）或全身化疗[n=56;环磷酰胺、多柔比星、长春新碱和泼尼松（CHOP）治疗（n=25）/长春新碱、环磷酰胺、多柔比星和泼尼松（VCAP）-多柔比星、雷莫司汀和泼尼松（AMP）-长春地辛、依托泊苷、卡铂和泼尼松（VECP）治疗（VCAP-AMP-VECP）或CHOP-VMMV疗法（n=31）]，并且显示中位生存期分别为16天和277天。在初始治疗后，对某些患者进行HSCT（n=6），从而显示三分之二（n=4）复发，三分之一（n=2）分别存活131天和203天。复发的ATL患者用常规挽救治疗（n=29）或抗CC趋化因子受体4抗体（mogamulizumab）（n=3）治疗。接受mogamulizumab治疗的患者分别表现出完全缓解（2）和部分缓解（1），持续时间短，分别为82天、83天和192天。根据ATL预后指数，接受化疗的患者（>5年）中，大多数显示为低风险和中等风险。由于诊断时患者年龄较大、接受姑息治疗的患者比例较高以及接受化疗和接受HSCT的长期存活者比例较小，ATL的总体存活率仍然较差。本研究阐述了目前的临床特点，治疗策略，并在临床实践中的结果。
Objective Despite the remarkable advances in chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT), adult T-cell leukemia-lymphoma (ATL) is still associated with a high mortality rate. It is therefore essential to elucidate the current features of ATL.Methods We retrospectively analyzed 81 patients with aggressive type ATL at our institution over a 7-year period based on Shimoyama's diagnostic criteria.Results Eighty-one patients with a median age of 67.5 years were classified as having acute (n=47), lymphoma (n=32), or chronic type (n=2) ATL. They were initially treated by either palliative therapy (n=25) or systemic chemotherapy [n=56; cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy (n=25)/vincristine, cyclophosphamide, doxorubicin, and prednisone (VCAP)-doxorubicin, ranimustine, and prednisone (AMP)-vindesine, etoposide, carboplatin, and prednisone (VECP) therapy (VCAP-AMP-VECP) or CHOP-VMMV therapy (n=31)], and showed median survival durations of 16 and 277 days, respectively. Subsequent to the initial treatment, HSCT (n=6) was performed for certain patients, thus revealing that two-thirds (n=4) relapsed, and one-third (n=2) survived for 131 days and 203 days, respectively.The relapsed ATL patients were treated with conventional salvage therapy (n=29) or anti-CC chemokine receptor 4 antibody (mogamulizumab) (n=3). The patients treated with mogamulizumab demonstrated complete response (2) and partical response (1) with short duration periods of 82 days, 83 days, and 192 days, respectively.Among the five long-term survivors (>5 years) who received chemotherapy, most showed a low and intermediate risk according to the ATL prognostic index.Conclusion In our study, the overall survival of ATL remains poor due to the advanced age of the patients at diagnosis, a high proportion of patients receiving palliative therapy, and a small proportion of long-term survivors receiving chemotherapy and undergoing HSCT. This study illustrates the current clinical features, treatment strategies, and outcomes in clinical practice.