Prochazka Vit, Faber Edgar, Raida Ludek, Papajik Tomas, Vondrakova Jana, Rusinakova Zuzana, Kucerova Ladislava, Myslivecek Miroslav, Indrak Karel
Department of Hemato-Oncology, University Hospital Olomouc, Czech Republic.
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2011 Mar;155(1):63-9. doi: 10.5507/bp.2011.009.
Peripheral T-cell lymphomas (PTCL) are infrequent subtypes of non-Hodgkin's lymphomas. The clinical course is aggressive and the median survival is about 2-3 years. An optimal first-line chemotherapy protocol has not been established and the role of high-dose therapy with autologous stem cell transplantation (ASCT) is still unclear.
To analyze the long-term outcome of unselected PTCL patients treated with intensive first-line chemotherapy with high-dose therapy and ASCT.
Here we report our experience with 29 patients with PTCL. The histological subtypes were as follows: peripheral T-cell lymphoma, not otherwise specified n=13; anaplastic large cell lymphoma (ALCL) ALK-negative n=5; ALCL ALK-positive n=3; ALCL with an unknown ALK status n=3; angioimmunoblastic lymphoma n=1; hepatosplenic lymphoma n=1; Sézary syndrome n=1; and enteropathy-associated T-cell lymphoma n=2. The median age at diagnosis was 48 years (29-64), most patients had advanced Ann Arbor stages (22 patients, 77%), IPI score ≥3 was found in 13 (45%) and PIT score ≥2 in 17 (59%) of the 29 patients. Eighteen patients received first-line high-dose therapy and autologous SCT consolidation; two patients were consolidaed with allogeneic SCT in the 1st complete remission and one patient in the 1st relapse. Ten patients with FDG-avid lymphoma were examined with integrated Positron emission tomography/ Computed tomography (PET/CT) at the time of diagnosis and after first-line therapy, two other patients were assessed with Positron emission tomography/ Computed tomography (PET/CT) only at time of restaging.
Nineteen (66%) patients achieved complete remission, 3 (10%) partial remission and 7 (24%) patients failed the treatment. The overall response rate was 76%. PET negativity (complete metabolic response) after therapy was achieved in 8/12 (75%) individuals. After a median follow-up of 55.1 months, 14 (48.3%) patients relapsed or progressed and nine patients died (lymphoma progression). Eleven patients (50% of chemosensitive patients) survived more than 50 months. Three of the long-term survivors were treated with allogeneic SCT. The 2-year event-free survival (EFS) was 52% (95% confidence interval [CI], 0.33-0.71); the 2-year overall survival (OS) rate reached 65% (95%CI, 0.47-0.84). PET negativity was associated with a lower probability of relapse (chi-square p=0.09).
Our data show that intensive first-line therapy with etoposide-doxorubicine-based regimens and ASCT consolidation may lead to long-term disease control in about a half of patients with chemosensitive PTCL. Achievement PET negativity is probably an essential prerequisite for long-term complete remission.
外周T细胞淋巴瘤(PTCL)是非霍奇金淋巴瘤中较少见的亚型。其临床病程具有侵袭性,中位生存期约为2至3年。尚未确立最佳的一线化疗方案,大剂量疗法联合自体干细胞移植(ASCT)的作用仍不明确。
分析未经选择的PTCL患者接受强化一线化疗、大剂量疗法及ASCT后的长期疗效。
我们报告了29例PTCL患者的治疗经验。组织学亚型如下:外周T细胞淋巴瘤,非特指型n = 13;间变性大细胞淋巴瘤(ALCL)ALK阴性n = 5;ALCL ALK阳性n = 3;ALK状态未知的ALCL n = 3;血管免疫母细胞性淋巴瘤n = 1;肝脾淋巴瘤n = 1;Sezary综合征n = 1;肠病相关T细胞淋巴瘤n = 2。诊断时的中位年龄为48岁(29 - 64岁),大多数患者Ann Arbor分期较晚(22例患者,77%),29例患者中有13例(45%)国际预后指数(IPI)评分≥3,17例(59%)预后指数(PIT)评分≥2。18例患者接受一线大剂量疗法及自体干细胞移植巩固治疗;2例患者在首次完全缓解期接受异基因干细胞移植巩固治疗,1例患者在首次复发时接受该治疗。10例氟代脱氧葡萄糖(FDG)摄取阳性的淋巴瘤患者在诊断时及一线治疗后接受了正电子发射断层扫描/计算机断层扫描(PET/CT)综合检查,另外2例患者仅在病情再分期时接受了PET/CT检查。
19例(66%)患者达到完全缓解,3例(10%)部分缓解,7例(24%)患者治疗失败。总缓解率为76%。治疗后8/12(75%)的患者达到PET阴性(完全代谢缓解)。中位随访55.1个月后,14例(48.3%)患者复发或病情进展,9例患者死亡(淋巴瘤进展)。11例患者(化疗敏感患者的50%)存活超过50个月。3例长期存活者接受了异基因干细胞移植治疗。2年无事件生存率(EFS)为52%(95%置信区间[CI],0.33 - 0.71);2年总生存率(OS)达到65%(95%CI,0.47 - 未找到完整内容)。PET阴性与较低的复发概率相关(卡方检验p = 0.09)。
我们的数据表明,基于依托泊苷 - 阿霉素方案的强化一线治疗及ASCT巩固治疗可能使约一半化疗敏感的PTCL患者实现长期疾病控制。实现PET阴性可能是长期完全缓解的必要前提。