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外周 T 细胞淋巴瘤:单一机构 208 例病例的组织学、分期和治疗反应分析。

Peripheral T-cell lymphomas: analysis of histology, staging and response to treatment of 208 cases at a single institution.

机构信息

Centre de lutte contre le cancer Institut Paoli Calmettes , Marseille , France.

出版信息

Leuk Lymphoma. 2013 Nov;54(11):2392-8. doi: 10.3109/10428194.2013.776680. Epub 2013 Mar 27.

Abstract

Peripheral T-cell lymphomas are characterized by a poor clinical outcome. We retrospectively analyzed 208 adults treated in our institution between 2000 and 2011. Median age at diagnosis was 55 years. Fifty-one percent had B symptoms and 51% serum elevated lactate dehydrogenase (LDH) levels. Eastern Cooperative Oncology Group (ECOG) performance status (PS) was 0-1 in 63% and 2-4 in 37%. According to Ann Arbor classification, 16% were at stage I-II and 84% at stage III-IV. Histological subtypes were: 39% peripheral T-cell non-Hodgkin lymphoma (NHL) unspecified (PTCL-U), 19.5% anaplastic large cell lymphoma (ALCL), with 9.5% ALK+ and 10% ALK-, and 25% angioimmunoblastic lymphoma (AILT). Primary extranodal lymphoma represented 17%, and 8% were diagnosed with hemophagocytosis. Induction chemotherapy was CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) in 87% of patients. The median number of chemotherapy cycles was 2 (1-7). A complete response was obtained in 57% of the patients. Among them, 32% had an autologous stem cell transplant (ASCT) and 10% allogeneic SCT, while 38% were primary refractory. Five-year overall survival (OS) was 28.5% (22.3-36.3), and 5-year event-free survival (EFS) was 18.4% (13.4-25.3). A multivariate analysis showed that ALCL-ALK+ (p = 0.004), AILT (p < 0.01), extranodal involvement (p = 0.001), PS > 1 (p = 0.04), LDH < normal (p = 0.003) and hemophagocytosis (p = 0.001) were independent adverse factors for OS. We conclude that conventional chemotherapy with intensive treatment is not sufficient to improve the response rate. Optimal management is required.

摘要

外周 T 细胞淋巴瘤的临床预后较差。我们回顾性分析了 208 例 2000 年至 2011 年在我院治疗的成年人患者。诊断时的中位年龄为 55 岁。51%有 B 症状,51%血清乳酸脱氢酶(LDH)水平升高。东部肿瘤协作组(ECOG)体能状态(PS)为 0-1 的占 63%,2-4 的占 37%。根据 Ann Arbor 分期,16%为Ⅰ-Ⅱ期,84%为Ⅲ-Ⅳ期。组织学亚型为:39%未特指外周 T 细胞非霍奇金淋巴瘤(PTCL-U),19.5%间变大细胞淋巴瘤(ALCL),其中 9.5%为 ALK+,10%为 ALK-,25%为血管免疫母细胞性 T 细胞淋巴瘤(AITL)。原发性结外淋巴瘤占 17%,8%诊断为噬血细胞性淋巴组织细胞增多症。87%的患者接受 CHOP(环磷酰胺、多柔比星、长春新碱、泼尼松)诱导化疗。化疗周期中位数为 2(1-7)个。57%的患者获得完全缓解。其中,32%接受了自体造血干细胞移植(ASCT),10%接受了异基因 SCT,而 38%为原发性耐药。5 年总生存率(OS)为 28.5%(22.3-36.3),5 年无事件生存率(EFS)为 18.4%(13.4-25.3)。多因素分析显示,ALK+间变大细胞淋巴瘤(p=0.004)、AITL(p<0.01)、结外累及(p=0.001)、PS>1(p=0.04)、LDH<正常值(p=0.003)和噬血细胞性淋巴组织细胞增多症(p=0.001)是 OS 的独立不良因素。我们得出结论,强化治疗的常规化疗不足以提高缓解率,需要优化治疗方案。

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