Therapies for Peripheral T-Cell Lymphomas

Abstract

Peripheral T-cell lymphomas (PTCLs) are a rare and heterogeneous group of disorders that, for the most part, are associated with a very poor prognosis. The standard therapy for PTCLs is CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or a comparable CHOP-like regimen that incorporates anthracyclines. With the exception of anaplastic lymphoma kinase-positive anaplastic large cell lymphoma (ALK ϩ ALCL), the cure rate for PTCLs with CHOP is low, and limited evidence suggests that anthracyclines do not improve the prognosis. However, there is no compelling evidence that any other regimen or approach is superior. It remains challenging to compare alternative therapies or treatment strategies with CHOP because the majority of data are retrospective and include diverse patient populations. Recently, prospective studies have been initiated exclusively for PTCL, and in some, select histologic subtypes are evaluated in an effort to remove heterogeneity. Encouragingly, there have been several new therapies emerging with activity in PTCLs and exciting novel combinations under consideration that will hopefully move the field forward and improve outcome in this challenging group of diseases.

Increased Expression of Phosphorylated FADD in Anaplastic Large Cell and Other T-Cell Lymphomas

“…Although ALK-positive ALCL is associated with a good prognosis,48 the same cannot be said for ALK-negative ALCL and PTCL-NOS, and improved therapies are urgently needed for these lymphomas 49–52. FADD and its interacting proteins, such as RIP1, are expressed in both ALK-negative and ALK-positive ALCL53 and may thus represent exciting therapeutic targets.…”

Treatment of High-Risk T-NHL with Stem Cell Transplantation: A Single Center Experience

“…Relapsed or refractory PTCLs are considered incurable under conventional therapy [1,3]. In consequence, different transplant approaches have been used as part of primary or salvage therapy of PTCL: high-dose therapy followed by autoSCT and allogeneic stem cell transplantation after myeloablative (MAC), reduced-intense (RIC) and minimal conditioning [4,9,21,22].…”

“…Conventional chemotherapy including anthracyclines leads to five-year overall survival rates of 30-40 % in the most common peripheral T-cell lymphoma entities [2,3]. Relapsed or refractory PTCLs are considered incurable under conventional therapy [1,3].…”

“…The 5-year survival rates ranges between 30 and 40 % in most trials including the most common entities like PTCL, nototherwise-specified (PTCL-NOS), systemic ALK-negative anaplastic large cell lymphoma (ALCL) and angioimmunoblastic T-cell lymphoma (AITL). Despite a lot of studies evaluating novel therapies convincing alternative treatment schedules are absent [2,3].…”

CD30 - the head of TNF-family… or a successful story of brentuximab vedotin

“…In ALK+ cases, the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy, with or without etoposide, leads to longtime survival in most of the patients. On the other hand, in ALK-, the CHOP achieves moderate results, so the autologous transplantation is the standard of treatment in the first line, and with such intensive approach, a five-year survival without the disease progression is 64%, which is longer than in other peripheral T-cell lymphomas (6).…”