open access

Vol 52, No 4 (2021)
Review article
Submitted: 2021-07-28
Accepted: 2021-07-28
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Treatment of peripheral T-cell lymphomas

Grzegorz Mazur1, Justyna Kanclerska1
DOI: 10.5603/AHP.2021.0064
·
Acta Haematol Pol 2021;52(4):334-339.
Affiliations
  1. Department of Internal Medicine, Occupational Diseases, Hypertension and Clinical Oncology, Wroclaw Medical University, Wrocław, Poland

open access

Vol 52, No 4 (2021)
REVIEW ARTICLE
Submitted: 2021-07-28
Accepted: 2021-07-28

Abstract

Peripheral T-cell lymphomas (PTCLs) are rare neoplasms that recently have been the subject of much research into their complex pathophysiology. PTCLs are a heterogeneous group of tumors consisting of nodal and extranodal leukemic and cutaneous neoplasms. PTCLs are associated with complex biology and arduous pathology which is currently being studied. According to this research, the pathophysiology of PTCLs can be divided into intrinsic and extrinsic mechanisms. Among the intrinsic mechanisms, scientists have described JAK-STAT pathway deregulation, as well as different somatic mutations including RB1, PTEN, TP53 and structural changes to the receptors. Also, there are scientific papers that correlate Epstein-Bárr virus or human T-cell lymphotropic virus type 1 infections with the occurrence of the neoplasm. PTCLs are most likely to develop in Asian and African populations. Due to poor clinical outcomes, PTCL treatment is the subject of intense clinical research. As a result of that, new drugs have been approved by the Food and Drug Administration for use among patients with refractory PTCL: pralatrexate, an antifolate drug; romidepsin, belinostat, an inhibitor for histone deacetylase, and brentuximab vedotin, a CD30 antibody. Also, clinical trials with mogamulizumab are being carried out for PTCL treatment. In addition to this, lenalidomide, as a substance that regulates the immune system and has shown antineoplastic effect in several hematological studies, could possibly be considered as treatment.

Abstract

Peripheral T-cell lymphomas (PTCLs) are rare neoplasms that recently have been the subject of much research into their complex pathophysiology. PTCLs are a heterogeneous group of tumors consisting of nodal and extranodal leukemic and cutaneous neoplasms. PTCLs are associated with complex biology and arduous pathology which is currently being studied. According to this research, the pathophysiology of PTCLs can be divided into intrinsic and extrinsic mechanisms. Among the intrinsic mechanisms, scientists have described JAK-STAT pathway deregulation, as well as different somatic mutations including RB1, PTEN, TP53 and structural changes to the receptors. Also, there are scientific papers that correlate Epstein-Bárr virus or human T-cell lymphotropic virus type 1 infections with the occurrence of the neoplasm. PTCLs are most likely to develop in Asian and African populations. Due to poor clinical outcomes, PTCL treatment is the subject of intense clinical research. As a result of that, new drugs have been approved by the Food and Drug Administration for use among patients with refractory PTCL: pralatrexate, an antifolate drug; romidepsin, belinostat, an inhibitor for histone deacetylase, and brentuximab vedotin, a CD30 antibody. Also, clinical trials with mogamulizumab are being carried out for PTCL treatment. In addition to this, lenalidomide, as a substance that regulates the immune system and has shown antineoplastic effect in several hematological studies, could possibly be considered as treatment.

Get Citation

Keywords

T-cell lymphoma, peripheral T-cell lymphoma, brentuximab vedotin, mogamulizumab

About this article
Title

Treatment of peripheral T-cell lymphomas

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 4 (2021)

Article type

Review article

Pages

334-339

DOI

10.5603/AHP.2021.0064

Bibliographic record

Acta Haematol Pol 2021;52(4):334-339.

Keywords

T-cell lymphoma
peripheral T-cell lymphoma
brentuximab vedotin
mogamulizumab

Authors

Grzegorz Mazur
Justyna Kanclerska

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