Karakas T, Bergmann L, Stutte H J, Jäger E, Knuth A, Weidmann E, Mitrou P S, Hoelzer D
Medical Clinic III, Hematology/Oncology, J.W. Goethe University, Frankfurt, FRG.
Leuk Lymphoma. 1996 Dec;24(1-2):121-9. doi: 10.3109/10428199609045720.
Peripheral T-cell lymphomas (PTCL) represent a heterogeneous group of T-cell malignancies including subentities with favourable (large cell anaplastic) or unfavourable (pleomorphic) prognosis. The clinical outcome of PTCL has been controversially discussed, but a worse prognosis than high-grade B-cell Non-Hodgkin's lymphomas (NHL) has been postulated by most authors. In this report we summarize the results of a prospective comparative study investigating the therapy outcome of 27 patients (pts) with PTCL and 55 pts. with high grade B-cell NHL and give an overview of therapy studies in PTCL. The histological subtypes were 14 pleomorphic, 8 large-cell anaplastic (Ki-1+), 2 angioimmunoblastic (AILD) and 3 other PTCL. In three patients the PTCL was associated with non-tropical sprue (11%). Nineteen patients presented with an advanced stage of disease (stage III and IV, 70%), 17 (63%) pts. had B-symptoms. The patients were treated with vincristine 2 mg d1, adriamycin 25 mg/m2 d1-3, cyclophosphamide 800 mg/m2 d1, prednisone 60 mg/m2 d1-7 and etoposide 120 mg/m2 d1-3 (VACPE). In 77% of pts. with PTCL and 84% of patients with high-grade B-cell NHL a complete remission (CR) was achieved. 75% of the complete responders with PTCL and 70% with B-NHL are still in ongoing CR. The subgroup of large-cell anaplastic attained a CR in 88%. The median observation time is 44 months (1(+)-77+). The probability of 1-, 3- and 5-year overall and disease-free survival for the T-cell group were 76%, 54%, 48% and 76%, 62%, 62%, respectively according to Kaplan-Meier. There was no significant difference regarding the remission rate, the overall-, event-free or disease-free survival compared to high-grade B-cell lymphomas. In conclusion, the VACPE regimen is an effective and feasible regimen in the management of PTCL achieving complete remissions in a large proportion of patients.
外周T细胞淋巴瘤（PTCL）是一组异质性T细胞恶性肿瘤，包括预后良好（大细胞间变性）或预后不良（多形性）的亚实体。PTCL的临床结局一直存在争议性讨论，但大多数作者推测其预后比高级别B细胞非霍奇金淋巴瘤（NHL）更差。在本报告中，我们总结了一项前瞻性比较研究的结果，该研究调查了27例PTCL患者和55例高级别B细胞NHL患者的治疗结局，并概述了PTCL的治疗研究。组织学亚型为14例多形性、8例大细胞间变性（Ki-1+）、2例血管免疫母细胞性（AILD）和3例其他PTCL。3例PTCL患者与非热带口炎性腹泻相关（11%）。19例患者表现为疾病晚期（III期和IV期，70%），17例（63%）患者有B症状。患者接受长春新碱2mg第1天、阿霉素25mg/m²第1 - 3天、环磷酰胺800mg/m²第1天、泼尼松60mg/m²第1 - 7天和依托泊苷120mg/m²第1 - 3天（VACPE）治疗。77%的PTCL患者和84%的高级别B细胞NHL患者实现了完全缓解（CR）。75%的PTCL完全缓解者和70%的B-NHL完全缓解者仍处于持续CR状态。大细胞间变性亚组的CR率为88%。中位观察时间为44个月（1（+）- 77+）。根据Kaplan-Meier法，T细胞组1年、3年和5年的总生存率和无病生存率分别为76%、54%、48%和76%、62%、62%。与高级别B细胞淋巴瘤相比，缓解率、总生存率、无事件生存率或无病生存率无显著差异。总之，VACPE方案是治疗PTCL的一种有效且可行的方案，在大部分患者中可实现完全缓解。
