Current Treatment Patterns and Outcomes in Patients with Peripheral T-Cell Lymphoma: Updated Results of the Nationwide, Multi-Center Prospective Registry Study (CISL 1404)
Authors: Cho Hyungwoo , Yoon Dok Hyun , Shin Dong-Yeop , Yoon Sung-Soo , Kim Seok Jin , Do Young Rok , Lee Gyeong-Won , Jae-Yong Kwak , Park Yong , Kim Min Kyoung , Kang Hye Jin , Yi Jun Ho , Yoo Kwai Han , Lee Won Sik , Byeong Bae Park , Jo Jae-Cheol , Eom Hyeon-Seok , Kim Hyo Jung , Seong Hyun Jeong , Won Young-Woong , Sohn Byeong Seok , Kwon Jihyun , Suh Cheolwon , Kim Won Seog 
Published: November 2019

Introduction
Peripheral T-cell lymphoma (PTCL) is a rare and heterogeneous group of diseases associated with poor prognosis, representing 10-15% of non-Hodgkin lymphomas. Although CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOP-like regimens are often preferred as 1st line treatment, the treatment outcome is poor with 5-year overall survival (OS) rate of 30-40%. In an effort to improve the survival outcomes of these patients, autologous hematopoietic stem cell transplantation (ASCT) as an upfront consolidative treatment has been proposed for patients achieving partial or complete remission after induction therapy. However, the role of ASCT still remains undefined since no randomized trials have demonstrated survival benefit of ASCT in this setting.
To better understand the clinical characteristics, treatment patterns, and outcomes in patients with PTCL, we have conducted a nationwide, multicenter, prospective registry study for newly diagnosed patients with PTCL.
Methods
Patients with PTCL receiving chemotherapy with curative intent were registered and prospectively monitored (ClinicalTrials.gov, no. NCT02364466). All patients were pathologically diagnosed with PTCL according to the 2008 World Health Organization classification of lymphoid neoplasms. Extranodal NK/T cell lymphoma, cutaneous T cell lymphoma, Mycosis fungoides and Sezary syndrome were excluded. The target number for enrollment was 200, and an interim analysis was previously reported at the time of enrollment of 155 patients (ASH 2017). An updated analysis of 198 patients was performed.
Results
The median age was 59 years (range, 49-70), 122 patients (61.6%) were male and 168 (84.9%) had ECOG performance status of 0-1. PTCL, not otherwise specified was the most common pathologic subtype (n = 80, 40.4%), followed by angioimmunoblastic T cell lymphoma (n = 60, 30.3%). The most frequently administered 1st line regimen was CHOP or CHOP-like regimen (n = 165, 83.3%), followed by ICE (ifosfamide, carboplatin, and etoposide) or ICE-like regimen (n = 23, 11.6%), and others (n = 10, 5.1%). With a median follow-up duration of 28.2 months (95% CI, 25.6-30.6), 2-yr progression-free survival (PFS) rate was 44.4% (95% CI, 37.5-57.4) and 2-yr OS rate was 64.4% (95% CI, 57.4-72.1).
Response evaluation for 1st line regimens were available in 175 patients. Among these patients, there was no significant difference in overall response rate (ORR) and complete response (CR) rate between patients treated with CHOP or CHOP-like vs. ICE or ICE-like regimen (ORR: 73.6 vs. 72.7%, P = 1.000; CR rate: 58.1% vs. 45.5%, P = 0.375). In addition, no significant difference was observed regarding PFS and OS between the two treatment groups (CHOP or CHOP-like vs. ICE or ICE-like; 2-yr PFS rate: 45.2 vs. 38.3%, P = 0.39; 2-year OS rate: 65.7 vs. 50.7% P = 0.43) (Figure 1A, B).
Among 121 patients younger than 65 years of age who are eligible for transplantation, autologous hematopoietic stem cell transplantation (ASCT) was performed as an upfront consolidative treatment in 51 patients (42.1%). Patients who received upfront ASCT was associated with significantly better PFS and OS compared with patients who did not, with a 2-yr PFS rate of 52.3 vs. 37.0% (P = 0.032) and 2-yr OS rate of 74.2 vs. 57.1% (P = 0.028), respectively (Figure 2A, B).
A total of 81 patients were treated with 2nd line chemotherapy for refractory or relapsed disease, and response evaluation for 2nd line chemotherapy was available in 63 patients. Among these patients ORR and CR rate were 49.2% and 30.2%, respectively.
Conclusion
Our study demonstrated that survival outcome with current treatment options for patients with PTCL remains poor. Although CHOP or CHOP-like regimens were the most commonly used 1st line regimens, no survival benefit was observed when compared with ICE or ICE-like regimens, suggesting that more efforts are needed to establish a standard 1st line treatment for PTCL. ASCT may provide survival benefit in transplant eligible patients, which warrants further evaluation in randomized controlled trials.

Disclosures
Yoon: Janssen: Consultancy; MSD: Consultancy; Novartis: Consultancy, Honoraria; Yuhan Pharma: Research Funding; Amgen: Consultancy, Honoraria; Genentech, Inc.: Research Funding; Kyowa Hako Kirin: Research Funding. Kim:F. Hoffmann-La Roche Ltd: Research Funding; Celltrion: Research Funding; Novartis: Research Funding; Donga: Research Funding; Kyowa-Kirin: Research Funding; Novartis: Research Funding; J + J: Research Funding.