Comprehensive analysis of peripheral T-cell and natural killer/T-cell lymphoma in Asian patients: A multinational, multicenter, prospective registry study in Asia

Abstract

Background: Peripheral T-cell lymphomas (PTCLs) are uncommon and their frequency is regionally heterogeneous. Several studies have been conducted to evaluate the clinical features and treatment outcomes of this disease entity, but the majority of these were conducted in limited areas, making it difficult to comprehensively analyze their relative frequency and clinical features. Furthermore, no consensus treatment for PTCLs has been established. Therefore, we conducted an Asia-specific study to understand the relative frequency of PTCLs and assess treatments and their outcomes in Asian patients.Methods: We performed a multinational, multicenter, prospective registry of adult patients with PT-CLs that was named as the International Cooperative non-Hodgkin T-cell lymphoma prospective registry study where thirty-two institutes from six Asian countries and territories (Korea, China, Taiwan, Singapore, Malaysia, and Indonesia) participated.Findings: A total of 486 patients were registered between April 2016 and February 2019, and more than a half of patients (57%) had stage III or IV. Extranodal natural killer (NK)/T-cell lymphoma was the most common subtype ( n = 139,28.6%), followed by angioimmunoblastic T-cell lymphoma (AITL, n = 120,24.7%), PTCL-not otherwise specified (PTCL-NOS, n = 101,20.8%), ALK-positive anaplastic large cell lymphoma (ALCL, n = 34,6.9%), and ALK-negative ALCL ( n = 30,6.2%). The median progression-free survival (PFS) and overall survival (OS) were 21.1 months (95% CI,10.6-31.6) and 83.6 months (95% CI, 56.7-110.5), respectively. Upfront use of combined treatment with chemotherapy and radiotherapy showed better PFS than chemotherapy alone in localized ENKTL whereas consolidation with upfront autologous stem cell transplantation (SCT) provided longer PFS in advance stage ENKTL. In patients with PTCLs other than ENKTL, anthracycline-containing chemotherapies were widely used, but the outcome of those regimens was not satisfactory, and upfront autologous SCT was not significantly associated with survival benefit, either. The treatment outcome of salvage chemotherapy was disappointing, and none of the salvage strategies showed superiority to one another.Interpretation: This multinational, multicenter study identified the relative frequency of each subtype of PTCLs across Asian countries, and the survival outcomes according to the therapeutic strategies currently used.

Extranodal T- and NK-cell lymphomas

“…With the exception of ENKTCL, which is the most common type of peripheral T-cell lymphoma (PTCL) in Asia, most of these diseases are rare [73,78]. Their identification and correct classification is of clinical importance as they differ in natural history, range from indolent to aggressive, and require different therapeutic approaches.…”

Immune Checkpoint Inhibitors in Peripheral T-Cell Lymphoma

“…There are 27 subtypes of PTCL according to the World Health Organization 2016 classification of lymphoid neoplasms ( Swerdlow et al, 2016 ). Of these, extranodal NK/T-cell lymphoma, nasal-type (ENKTL), which is closely associated with Epstein-Barr virus (EBV) infection, is the most common in East Asian countries ( Yoon et al, 2021 ). Other common subtypes of PTCL include PTCL, non-specific (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), and anaplastic lymphoma kinase (ALK)+/− anaplastic large cell lymphoma (ALCL) ( Swerdlow et al, 2016 ; Liu et al, 2020 ).…”

Impact of Epstein-Barr Virus on Peripheral T-Cell Lymphoma Not Otherwise Specified and Angioimmunoblastic T-Cell Lymphoma

“…Peripheral T-cell lymphomas (PTCLs) are neoplasms derived from the post-thymic lymphocytes and account for approximately 15% of non-Hodgkin lymphoma in the Western hemisphere ( 1 ). Angioimmunoblastic T-cell lymphoma (AITL) and PTCL not otherwise specified (PTCL-NOS) are the second and third most common PTCLs in Asia and account for 24.7% and 20.8% of cases, respectively ( 2 ).…”