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Nodal and extranodal peripheral T/NK-cell neoplasms: Current aspects

By
Ivan Petković Orcid logo ,
Ivan Petković
Aleksandar Popović ,
Aleksandar Popović
Miljana Džunić ,
Miljana Džunić
Ivica Pejčić
Ivica Pejčić

Abstract

Peripheral T/NK–cell lymphomas (PTCL) are rare, bizarre, and extremely diverse cancers. The disease is prone to relapse with a high level of chemorefractoriness leading to a poor outcome. Almost 70% of patients will experience relapse, with a median 5–year overall survival (OS) in approximately 30%. Upfront management of PTCL has been extrapolated from the treatment paradigm for aggressive B–NHL. However, universally accepted induction is rather palliative than curative. Regardless of the maximal reinforcement of upfront management, only event-free survival has been influenced but not the OS. All actual guidelines emphasize the importance of the autologous stem cell transplantation (auto–SCT) as a consolidation of first major response. The allogeneic SCT (allo–SCT) is not evidence–based part of upfront management. Nevertheless, its use is justified in the relapsed/refractory setting. Unfortunately, the vast majority of patients are not candidates for aggressive treatment modalities making the recommended paradigm as limited feasible. Regarding such a situation, novel compounds are warranted. Although presented data indicate ominous prognosis in PTCL, it is important to denote that there has been evidence – based improvement in the treatment paradigm by the introduction of L–Asparaginase and targeted therapy for CD30+ PTCL. In this sense, a considerable number of new compounds entered early phase trials and gave promising results. All lights have been focused on upcoming results given the fact that at the moment we have not much to offer to the patients who have relapsed or were primary refractory. 

References

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Citations

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2

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Ivan Petković, Michele Ritucci, Ana Stojković, Slavica Stojnev, Aleksandar Popović, Irena Conić, Milica Radić, Miljana Džunić, Miljan Krstić

(2025)

The Significance of the Microenvironment in T/Nk-Cell Neoplasms

International Journal of Molecular Sciences, 26(22)

10.3390/ijms262211225

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Ivan Petković, Slavica Stojnev, Aleksandar Popović, Miljan Krstić, Ivica Pejčić

(2021)

EBV Negative Angioimmunoblastic T–Cell Lymphoma with Sequential Development of Diffuse Large B–Cell Lymphoma in Course of Progression

Indian Journal of Hematology and Blood Transfusion, 37(4)

10.1007/s12288-021-01425-w

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