Volumen: 20 # Number : Número Extraordinario
Publication Date : Agosto Year: 2016
The T-Cell Project: lessons from
observational prospective studies
Authors: Bellei M, Chiattone C, Pavlosky A,
Gabus R, Cabrera M E, Federico M
Abstract: Peripheral T-cell and NK-cell lymphomas (PTCLs)
are a group of diverse lymphoproliferative disorders
including very different entities with diverse
biological and clinical behavior. The updated version
of the WHO classification provided significant
advances in both nodal and extranodal mature T-cell
and NK-cell lymphoma, which have led to the introduction
of new provisional entities, mainly thanks
to the results of the genomic studies that have been
performed since the 4th edition was published in
2008. However, these new information have not
translated so far in clinical practice and remain restricted
to clinical trials. Prognosis of PTCLs has
been extensively studied by means of retrospective
evaluations. Since these kind of studies carry on biases,
the International T-Cell Lymphoma Project,
after its retrospective study on 1,314 patients, promoted
in 2006 the T-Cell Project, an observational
prospective registry aiming at improving information
on prognosis of the two most frequent subtypes
of PTCLs (peripheral T-cell lymphoma, not
otherwise specified and angioimmunoblastic T-cell
lymphoma) and to get better insight the clinical an
biological characteristics of other rarer nodal and extranodal PTCLs. The T-Cell project is a well consolidated
network having at present 74 participating
sites from four regional areas.
The prognosis for PTCLs remains poor, mainly due
to low response rates to induction therapy, to the
short duration of response with standard combination
chemotherapy regimens and to the controversial
role of transplant as consolidation after achievement
of response with first line therapy. However,
in the recent years a number of novel agents have
been approved for the treatment of PTCLs in the relapsed/
refractory setting, and some trials have been
opened for this subset of patients. Moreover, in the
last decade advances in the biological and genetic
characteristics of these diseases have been made,
and some other are planned or ongoing. The biobank
of the T-Cell Project and the wide cooperation in it
of a number of institutions could serve for future
studies on developing trials specific for the different
subtypes for the first line treatment exploring novel
combinations in front line approaches.
Key words: T-cell lymphomas;
prognosis;
treatment.
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