Mature T-Cell Lymphomas (PTCLs) represent a heterogeneous group of haematological malignancies, with a fairly poor outcome. Due to their rarity, PTCLs are very poorly understood and information useful to develop more rational therapeutic approaches and substantially improve the prognosis are limited. The T-Cell Project (TCP), launched a prospective collection of accurate data coming from patients with newly diagnosed PTCLs, with the aim of improving knowledge on these rare diseases. From Sept 2006 to Jan 2016, 1,439 cases have been registered by 74 Institutions world-wide. PTCL-NOS emerged as the most frequent subtype (36%). Combination chemotherapy was the preferred approach (90%), anthracycline-containing regimens being the favourite (84%). Consolidative ASCT was reported in 7%, with different geographic distribution. After induction therapy 54% achieved a CR and 18% a PR. After a median follow-up of 35 months, 5-yr OS and PFS were 44% and 33%, respectively. The ALCL, ALK+ showed the best 5-yr OS (73%). The TCP is the largest ongoing prospective registry; and is now moving forward to the establishment of a large biorepository. This exceptional position could allow to build future treatment platforms predicated on our biological understanding of the disease, which we anticipate will lead to the development of subtype specific treatments.
T-cell lymphomas: where we are and where we a re moving forward / Bellei, Monica; Conte, Luana; Tarantino, Vittoria; Federico, Massimo. - In: DRUGS AND CELL THERAPIES IN HEMATOLOGY. - ISSN 2281-4876. - STAMPA. - 3:3(2015), pp. 176-186.
T-cell lymphomas: where we are and where we a re moving forward
BELLEI, Monica;Tarantino, Vittoria;FEDERICO, Massimo
2015
Abstract
Mature T-Cell Lymphomas (PTCLs) represent a heterogeneous group of haematological malignancies, with a fairly poor outcome. Due to their rarity, PTCLs are very poorly understood and information useful to develop more rational therapeutic approaches and substantially improve the prognosis are limited. The T-Cell Project (TCP), launched a prospective collection of accurate data coming from patients with newly diagnosed PTCLs, with the aim of improving knowledge on these rare diseases. From Sept 2006 to Jan 2016, 1,439 cases have been registered by 74 Institutions world-wide. PTCL-NOS emerged as the most frequent subtype (36%). Combination chemotherapy was the preferred approach (90%), anthracycline-containing regimens being the favourite (84%). Consolidative ASCT was reported in 7%, with different geographic distribution. After induction therapy 54% achieved a CR and 18% a PR. After a median follow-up of 35 months, 5-yr OS and PFS were 44% and 33%, respectively. The ALCL, ALK+ showed the best 5-yr OS (73%). The TCP is the largest ongoing prospective registry; and is now moving forward to the establishment of a large biorepository. This exceptional position could allow to build future treatment platforms predicated on our biological understanding of the disease, which we anticipate will lead to the development of subtype specific treatments.File | Dimensione | Formato | |
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