ACTA FAC MED NAISS 2020;37(2):99-120|
ACTA FAC MED NAISS 2020;37(2):99-120 |
Review article
UDC:
616.428-006.44-08
DOI: 10.5937/afmnai37-24708
Nodal and Extranodal Peripheral T/NK-Cell Neoplasms: Current Aspects
Ivan Petković1,2, Aleksandar Popović2, Miljana Džunić2,
Ivica Pejčić1,2
2Oncology
Clinic, Clinical Center Niš, Niš, Serbia
SUMMARY
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.
Key words:
NK/T cell lymphoma, nodal, extranodal, treatment, new agencies