How I manage peripheral T-cell lymphoma, not otherwise specified and angioimmunoblastic T-cell lymphoma: current practice and a glimpse into the future.

Details

Serval ID
serval:BIB_5113D39545AE
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
How I manage peripheral T-cell lymphoma, not otherwise specified and angioimmunoblastic T-cell lymphoma: current practice and a glimpse into the future.
Journal
British journal of haematology
Author(s)
Schmitz N., de Leval L.
ISSN
1365-2141 (Electronic)
ISSN-L
0007-1048
Publication state
Published
Issued date
03/2017
Peer-reviewed
Oui
Volume
176
Number
6
Pages
851-866
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Abstract
Peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS) and angioimmunoblastic T-cell lymphoma (AITL) are the most frequent of more than 20 mature PTCL entities featuring a broad spectrum of morphological, immunophenotypic, molecular and clinical characteristics. Unfortunately, recent progress in understanding the (epi)genetic background of PTCL has not been met with similar advances in treatment. Thus, CHO(E)P [cyclophosphamide, doxorubicin, vincristine, and prednisone (plus etoposide)] remains standard first-line therapy. Patients without comorbidities achieving complete or partial remission proceed to autologous stem cell transplantation. With this approach about 50% of patients survive long-term. Patients relapsing after or progressing during first-line therapy have a dismal prognosis. They receive salvage gemcitabine-therapy followed by allogeneic transplantation whenever possible. After allografting, approximately half of the patients survive long-term; any other treatment is palliative. New drugs investigated in phase II studies achieved response rates between 10% and 30%; long-term remissions are the exception to the rule. While most new drugs are not licensed and not readily available, a plethora of other innovative drugs targeting (epi-)genetic abnormalities are in early development. These, together with combinations of new and old drugs, will hopefully increase response to first-line therapy, bridge more patients to transplantation, and finally improve prognosis for all patients with PTCL.

Keywords
Combined Modality Therapy/adverse effects, Combined Modality Therapy/methods, Diagnosis, Differential, Disease Management, Drug Discovery, Drug Resistance, Neoplasm, Humans, Immunoblastic Lymphadenopathy/diagnosis, Immunoblastic Lymphadenopathy/therapy, Lymphoma, T-Cell, Peripheral/diagnosis, Lymphoma, T-Cell, Peripheral/etiology, Lymphoma, T-Cell, Peripheral/therapy, Molecular Targeted Therapy, Neoplasm Staging, Practice Patterns, Physicians', Prognosis, Recurrence, Standard of Care, Treatment Outcome
Pubmed
Web of science
Open Access
Yes
Create date
20/12/2016 9:00
Last modification date
20/08/2019 14:06
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