Abstract
Simple SummaryThe intent of this paper is to critically revise the epidemiology, etiology, main clinical features, histopathology, immunophenotype, molecular characteristics, prognosis, and therapeutic options of peripheral T-cell lymphoma, not otherwise specified (PTCL_NOS). PTCL_NOS represents the commonest category of PTCL. Regarded as an exclusion diagnosis, it has a quite distinctive profile, based on the authors’ experience and data from the literature. New therapeutic agents are discussed, which might improve the prognosis of this neoplasm that remains dismal based on conventional chemotherapy.Peripheral T-cell lymphoma, not otherwise specified (PTCL_NOS) corresponds to about one fourth of mature T-cell tumors, which overall represent 10–12% of all lymphoid malignancies. This category comprises all T-cell neoplasms, which do not correspond to any of the distinct entities listed in the WHO (World Health Organization) Classification of Tumours of Haematopoietic and Lymphoid Tissues. In spite of the extreme variability of morphologic features and phenotypic profiles, gene expression profiling (GEP) studies have shown a signature that is distinct from that of all remaining PTCLs. GEP has also allowed the identification of subtypes provided with prognostic relevance. Conversely to GEP, next-generation sequencing (NGS) has so far been applied to a limited number of cases, providing some hints to better understand the pathobiology of PTCL_NOS. Although several pieces of information have emerged from pathological studies, PTCL_NOS still remains a tumor with a dismal prognosis. The usage of CHOEP (cyclophosphamide, doxorubicin, vincristine, prednisone, etoposide) followed by autologous stem cell transplantation may represent the best option, by curing about 50% of the patients whom such an approach can be applied to. Many new drugs have been proposed without achieving the expected results. Thus, the optimal treatment of PTCL_NOS remains unidentified.
Highlights
Peripheral T-cell lymphomas (PTCLs) correspond to a heterogeneous group of neoplasms arising from mature, post-thymic ( “peripheral”) T-lymphocytes [1]
Peripheral T-cell lymphoma, not otherwise specified (PTCL_NOS) corresponds to about one fourth of mature T-cell tumors, which overall represent 10–12% of all lymphoid malignancies. This category comprises all T-cell neoplasms, which do not correspond to any of the distinct entities listed in the WHO (World Health Organization) Classification of Tumours of Haematopoietic and Lymphoid Tissues
This review focuses on the pathobiology, clinics, and therapeutic perspectives of PTCL_NOS, aiming to assist the reader in problem solving and decision making in such a complex field of haemato-oncology
Summary
Peripheral T-cell lymphomas (PTCLs) correspond to a heterogeneous group of neoplasms arising from mature, post-thymic ( “peripheral”) T-lymphocytes [1]. The World Health Organization (WHO) Classification of Tumors of Haematopoietic and Lymphoid Tissues divides PTCLs into nodal, extranodal, and leukemic types, each including multiple distinct disease entities [1]. Neoplasms other than angioimmunoblastic T-cell lymphoma (AITL) but showing a T-follicular helper (TFH) profile, which in the past had been included in the PTCL_NOS chapter, were moved to the new group of nodal peripheral T-cell lymphomas of TFH origin The latter is characterized by a distinctive morphology (small-medium sized cells with clear cytoplasm), expression of at least two but preferably three TFH-associated markers (among BCL6, CD10, PD1/CD279, ICOS/CD278, SAP, CXCL13, and CCR5), gene expression profile, and mutational landscape [2,3,4]. This review focuses on the pathobiology, clinics, and therapeutic perspectives of PTCL_NOS, aiming to assist the reader in problem solving and decision making in such a complex field of haemato-oncology
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have