The Clinical Features and Outcomes of Peripheral T-Cell Lymphomas, Not Otherwise Specified (PTCL-NOS): A Single Institution Retrospective Analysis of 30 Cases
Authors: Guo Zijing , Ma Mengxue , Xing Lina , Liu Yao , Qiao Shukai 
Published: December 2024

Background: PTCL-NOS is the most common histological subtype of PTCL, characterized by high heterogeneity in clinical presentation, immunophenotype, and genetic features. The incidence of PTCL-NOS is regional distributed, with PTCL accounting for 23-27% of non-Hodgkin lymphomas in China, significantly higher than the 10% in Western countries. In terms of treatment, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) like regimens are still the first-line therapy; However, due to the high invasiveness and recurrence rate of the disease, the five-year progression free survival (PFS) rate is only 29%. In recent years, with the application of new drugs such as histone deacetylase (HDAC) inhibitor Chidamide and antibody conjugated drugs brentuximab-vedotin (BV) etc, certain clinical benefits have been brought to PTCL patients, however these patients still faces significant survival challenges.

Aim:The aim of this study was to explore the clinical features of Chinese patients with PTCL-NOS in a single institution, to identify potential prognostic factors, and to provide references for early diagnosis and improvement of long-term survival

Methods: Thirty patients with treatment-naïve PTCL-NOS admitted to the Second Hospital of Hebei Medical University from January 2015 to 2023 were analyzed retrospectively.

Results: A total of 30 patients were included.The median age of the patients at diagnosis was 59 years old (range: 17~70) , and the male to female ratio was 2.75:1. Most patients were an advanced stage at diagnosis, with intranodal primary as the main type; in terms of immunophenotype, there was a variable loss of pan-T cell markers, most commonly seen in abnormal expression of CD5 and CD7. The CD30 positivity rate in this study was 53.8%, which is higher than previous literature reports, or may be related to the different cutoff values of CD30 positive and a relatively small sample size of this study. This suggests that these patients with CD30 positive can use BV as a preferential therapy choice.The follow-up period ended in September 2023, with a median follow-up time of 59.0 months. The 3-year OS and PFS were 50.4% and 28.9%, respectively. The overall 5-year overall survival (OS) and 5-year PFS were 44.8% and 21.7%, respectively. Multivariate analysis showed that ECOG score &gt;1, bone marrow involvement, and PLT &lt;150×109/L were independent risk factors for OS, while bone marrow involvement and ALB &lt;35g/L were independent risk factors for PFS. In terms of treatment, there was no significant difference between the CHOPE regimen and the CHOP regimen, and the combination with Chidamide showed a trend towards improved OS and PFS. Two relapsed and refractory patients wtith CD30 positive had received salvage chemotherapy based on BV monoclonal antibody and achieved complete metabolic remission, followed by sequential allogeneic hematopoietic stem cell transplantation and obtained long-term sustained remission.

Conclusion: Patients with PTCL-NOS in China have a poor prognosis, and the conventional CHOP-like regimens has limited efficacy. Several novel drugs such as Chidamide and BV may improve patient prognosis in some individual patients. Future research should focus on the development of effective clinical prediction tools and treatment strategies, particularly accelerating the development of novel drugs for PTCL