Symptoms and General Pathology

All Conditions

Neoplasms

Blood and Lymph Conditions

Immune System Diseases

Rare Diseases

Lymphoma

Lymphoma, T-Cell

Lymphoma, T-Cell, Peripheral

Neoplasms by Histologic Type

Lymphoproliferative Disorders

Lymphatic Diseases

Immunoproliferative Disorders

Lymphoma, Non-Hodgkin

Lymphosarcoma

Peripheral T-cell Lymphoma

Martina Manni, MSc, PhD

Monica Civallero, MSc, PhD

Ranjana Advani, MD

Jessica Catherine Lam, MSc, PhD

Attilio Guarini, MD

Angela Monica Sciacovelli, PhD

Maurizio Musso, MD

Emilio Ianitto, MD

Anna Marina Liberati, MD

Viviana Appolloni, PhD

Ciprian Tomuleasa, MD

Catalin Vlad, MD

Iryna Kriachok, MD

Tetiana Skrypets, MD

Massimo Federico, MD

Julie Vose, MD

Steven Horwitz, MD

Miles Prince, MD

Kim Won Seog, MD

Dolores Caballero, MD

Francesco Zaya, MD

Stefano Luminari, MD

Andrei Shustov, MD

Pierluigi Porcu, MD

Astrid Pavlovsky, MD

Carlos Chiattone, MD

Francine Foss, MD

Christopher Fox, MD

This study T-Cell Project 2.0 is based on the former International PTCL study designed by the International T-cell Non-Hodgkin's Lymphoma Study Group (T-Cell Project 1.0: Prospective Collection of Data in Patients With Peripheral T-Cell Lymphoma) as a prospective collection of data to predict the prognosis of patients with the more frequent subtypes of PTCL. It is a prospective, longitudinal, international, observational study of patients with newly diagnosed peripheral T-cell lymphoma aiming to verify whether this prospective collection of data would allow achieving a more accurate information on T-cell lymphomas.

The study aims to better define the clinical relevance of the new WHO Classification, the role of FDG-PET in staging and response assessment, the prognosis of different entities, the genomic landscape of different subtypes, and to investigate on most optimal treatment strategies for these neoplasms in the real-world population as well as molecular markers and to explore the prognostic or predictive implications of them in PTCL.

The study aims to better define the clinical relevance of the new WHO Classification, the role of FDG-PET in staging and response assessment, the prognosis of different entities, the genomic landscape of different subtypes, and to investigate on most optimal treatment strategies for these neoplasms in the real-world population.

Peripheral T-cell non-Hodgkin lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorder arising from mature T cells of post-thymic origin at different stages of differentiation with different morphological patterns, phenotypes, and clinical presentation. All subtypes are found more commonly in male patients, and the median age at diagnosis is 62 years. This disease is generally associated with high relapse rates and a poor prognosis, with inferior treatment outcomes compared with B-cell lymphomas and have a 5-year-survival \< 32%.

T-cell lymphomas are widely recognized as a complex and heterogeneous group of lymphoproliferative disorders, generally associated with high relapse rates and a poor prognosis. Because of their rarity, they are still very poorly understood.

The introduction of new and more effective therapies and better technologies led the International T-cell non-Hodgkin's Lymphoma Study Group to launch the T-cell Project 2.0 in order to have a contemporary, real-time understanding of the T-cell lymphoma biology and treatment, together with the application of contemporary technologies to further identification of new therapeutic targets.

Per protocol, patients are evaluated according to the treating physician's standard practice. There are no specific evaluations or visits required for the Registry. Data captured in the Registry reflects what is routinely collected for patients with PTCL.

The study plans to collect the tissue sample for central review. The ordinary fixation, cryopreservation and routine tumor cytogenetics are planned for biopsy samples. Chairmen of the Histopathology Review Panel will locate Regional sites where expert hematopathologists will review the material and perform a panel of immunostains (T-cell panel + CD20) and markers not assessed at local site.

Adding of blood sample collection will allow estimating prospectively the frequency of pEBVd detection in our cohort of PTCL patients at baseline and at the end of initial therapy, to characterize agreement between pEBVd and EBER in tumor tissue, and to explore the prognostic or predictive implications of detectable pEBVd in PTCL. Finally, to investigate the genetics and pathogenic mechanisms of aggressive PTCLs on an international scale.

Formalin-fixed tissue for central diagnostic pathology review; Peripheral blood samples.

Prospective Observational International Registry of Patients With Newly Diagnosed Peripheral T Cell Lymphoma.

We plan to share with other involved researchers the minimum information on IPD for publication.

Measured from the date of diagnosis until the date of disease progression or death from T-cell Lymphoma

Measured from the date of diagnosis until death from any cause

Measured from the date of diagnosis until the date of event

Complete response rate at 30 months (CR30) after enrollment (i.e., initiation of treatment)

Associazione Angela Serra per la ricerca sul cancro

Digestive System Diseases

Skin and Connective Tissue Diseases

Intestinal Diseases

Panniculitis

Julie M. Vose, MD

Emanuele Zucca, MD

Joseph M Connors, MD

Steven M. Horwitz, MD

Francine M. Foss, MD

Pier Luigi Zinzani, MD

Silvia Montoto, MD

Aaron Polliack, MD

Stefano A. Pileri, MD

Young H. Ko, MD

The designed study follows up the retrospective previous one by the International T-cell Non-Hodgkin's Lymphoma Study Group (International Peripheral T-Cell Lymphoma Project).

It is designed as a prospective collection of information potentially useful to predict the prognosis of newly diagnosed patients with the more frequent subtypes of Peripheral T-cell lymphoma (Peripheral T-cell lymphoma unspecified and Angioimmunoblastic T-cell lymphoma) and to better define clinical characteristics and outcome of the more uncommon subtypes

Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of neoplasms that are derived from post-thymic lymphoid cells at different stages of differentiation with different morphological patterns, phenotypes, and clinical presentations. PTCLs are highly diverse, reflecting the diverse cells from which they can originate. Peripheral T-Cell Lymphomas account for 5-10% of all lymphoproliferative disorders in the Western hemisphere, with an overall incidence of 0.5-2 per 100,000 per year, and have a striking epidemiological distribution, with higher incidence in Asia.

The clinical features of PTCLs are extremely heterogeneous. PTCLs express even more clinical diversity than B-cell NHLs, and there is a close, though not absolute, relationship between some unusual clinical features and certain histological subtypes. Despite efforts to transferring to patients with T-cell lymphomas the most recent advances in the treatment of other subtypes of B-cell lymphomas, the prognosis of patients with PTCL is still poor an, unfortunately, the optimal therapy for PTCL is still unknown. The complete response rate is rather low, ranging from 40% to 50% with a median Relapse Free Survival (RFS) of 2-3 years. As a consequence of the aggressiveness of the disease and of the low efficacy of available salvage treatments, Overall Survival (OS) is also short and the long-term survival rate is lower than 10% in many series.

To better define the clinical outcome of PTCL-NOS, the Intergruppo Italiano Linfomi (IIL, now Fondazione Italiana Linfomi, FIL) performed a large study on 385 patients diagnosed and treated in the 1990s and defined a prognostic model specifically devised for patients with this uncommon disease (Gallamini, A. et al Blood, 2004. 103(7): p. 2474-9). In addition to defining a prognostic model specifically devised for PTCL-NOS, the FIL study confirms the relevance of research on series of clearly defined cases in order to the development of rationally designed and potentially more-efficacious treatment modalities. More recently, the role of biological features of the disease is emerging as an important issue not only for understanding its pathogenesis but also for prognosis and for addressing specific biologic targets altered in the neoplasia. Significant progress in the prognosis of PTCL can be expected from the novel, sophisticated, and powerful technologies of genomics and proteomics, which will allow more reliable subtyping of PTCL into distinct clinical groups characterized by different patterns of survival, as already demonstrated for some B-NHLs.

One common limitation of existing studies on prognosis of PTCL is their retrospective nature. Currently available data are based on analysis performed on series collected over a long period of time. This aspect is very important as it may introduce relevant biases in the collected series. First classification systems have changed dramatically over time and cases may have been defined in differently based on diagnosis year. Second some clinical or laboratory data which now are considered as prognostic relevant may have not been determined in older series of patients. Third in a retrospective analysis there is no guarantee that collected series are based on real consecutive cases. These are the reasons why we thought it would be useful to start a new study based on the prospective registration in a short period of time of patients with diagnosis of Peripheral T-cell lymphoma for whom it would be possible collect an exhaustive set of clinical data and biological information.

Formalin-fixed tissue for central diagnostic pathology review

T-Cell Project: Prospective Collection of Data in Patients With Peripheral T-Cell Lymphoma

Prospective Collection of Data in Pts With Peripheral T-Cell Lymphoma: PTCL,NOS;AITL; Extranodal NK/T-cell;Enteropathy-type; Hepatosplenic γ-δ; Subcutaneous Panniculitis-like; ALCL,Primary Systemic Type. By the Intl. T-Cell Lymphoma Project

Preliminary analysis results will be made available during the study on the single population and separately for each subtype. Final results will be made available 6-12 months after the end of the study

Fondazione Italiana Linfomi - ETS

INTERNATIONAL T-CELL NON-HODGKIN'S LYMPHOMA STUDY GROUP

Study title	NCT ID	Conditions	Eligibility	Organization
Prospective Observational International Registry of Patients With Newly Diagnosed Peripheral T Cell Lymphoma.	NCT03964480	Peripheral T-Ce...	18 Years -	Associazione Angela Serra per la ricerca sul cancro
T-Cell Project: Prospective Collection of Data in Patients With Peripheral T-Cell Lymphoma	NCT01142674	Lymphoma, T-Cel...	18 Years -	Associazione Angela Serra per la ricerca sul cancro
Prospective Observational International Registry of Patients With Newly Diagnosed Peripheral T Cell Lymphoma.	NCT03964480	Peripheral T-Ce...	18 Years -	Associazione Angela Serra per la ricerca sul cancro
Prospective Observational International Registry of Patients With Newly Diagnosed Peripheral T Cell Lymphoma.	NCT03964480	Peripheral T-Ce...	18 Years -	Associazione Angela Serra per la ricerca sul cancro

Spots Global Cancer Trial Database for biological characteristics