Ellin Fredrik, Jerkeman Mats, Hagberg Hans, Relander Thomas
Department of Internal Medicine, Kalmar County Hospital , Kalmar , Sweden.
Acta Oncol. 2014 Jul;53(7):927-34. doi: 10.3109/0284186X.2014.889850. Epub 2014 Mar 10.
T-cell lymphoblastic lymphoma (T-LBL) is a rare neoplasm of precursor lymphoblast origin, for which there is no standard treatment for adults. Results of current treatment strategies in selected populations do exist but are largely unreported for unselected series. Here, we aimed to investigate treatment outcome in a population-based cohort.
Patients were identified through the Swedish Lymphoma Registry and data was retrospectively collected for all adult (≥ 18 years) Swedish T-LBL patients diagnosed during 2000-2009.
A total of 39 patients with median age 40 years (range 18-78) were identified with females being significantly older than males (median age 66 vs. 37, p = 0.027). The five-year overall survival for all patients was 42%. Female gender was associated with shorter survival also when adjusted for treatment strategy and age [hazard ratio (HR) 4.29; p = 0.002]. Thirty patients received intensive chemotherapy, otherwise used for treatment of acute lymphoblastic leukemia (ALL), which resulted in an overall response rate of 97% and a five-year progression-free survival (PFS) of 49%. In this group only CNS involvement at diagnosis predicted shorter PFS (HR 13.3; p = 0.03). Among patients treated with hyper-CVAD the addition of mediastinal irradiation resulted in prolonged time to progression compared to patients receiving only chemotherapy (p = 0.047). The major reason for treatment failure was relapse and in this series 18-fluoro-deoxyglucose positron emission tomography (PET) did not predict this risk.
This population-based study indicates that all fit T-LBL patients should be considered for intensive treatment. Our results also suggest a beneficial effect of mediastinal irradiation in combination with hyper-CVAD treatment. Relapsing patients have a dismal outcome irrespective of salvage treatment.
T 细胞淋巴母细胞淋巴瘤（T-LBL）是一种起源于前体淋巴母细胞的罕见肿瘤，目前尚无针对成人的标准治疗方案。现有部分特定人群的当前治疗策略结果，但对于未经筛选的病例系列，相关报道大多缺失。在此，我们旨在调查一个基于人群的队列的治疗结果。
通过瑞典淋巴瘤登记处识别患者，并回顾性收集 2000 年至 2009 年期间确诊的所有成年（≥18 岁）瑞典 T-LBL 患者的数据。
共识别出 39 例患者，中位年龄 40 岁（范围 18 - 78 岁），女性明显比男性年龄大（中位年龄 66 岁对 37 岁，p = 0.027）。所有患者的五年总生存率为 42%。在调整治疗策略和年龄后，女性性别仍与较短生存期相关[风险比（HR）4.29；p = 0.002]。30 例患者接受了强化化疗，该化疗方案常用于治疗急性淋巴细胞白血病（ALL），总体缓解率为 97%，五年无进展生存率（PFS）为 49%。在该组中，仅诊断时存在中枢神经系统受累预示着较短的 PFS（HR 13.3；p = 0.03）。在接受 Hyper-CVAD 治疗的患者中，与仅接受化疗的患者相比，加用纵隔放疗可延长疾病进展时间（p = 0.047）。治疗失败的主要原因是复发，在本系列中，18 - 氟 - 脱氧葡萄糖正电子发射断层扫描（PET）无法预测这种风险。
这项基于人群研究表明，所有适合的 T-LBL 患者均应考虑接受强化治疗。我们的结果还提示纵隔放疗联合 Hyper-CVAD 治疗具有有益效果。复发患者无论接受挽救性治疗，预后都很差。
