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

Обзор

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.

Подробное описание

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.

Первичные конечные точки

• Progression-free survival (PFS) [Срок оценки: 2 year]

Вторичные конечные точки (4)

• Overall Survival (OS) [Срок оценки: 3 and 5 year]
• Progression-Free Survival (PFS) [Срок оценки: 3 and 5 years]
• Event Free Survival (EFS) [Срок оценки: at 24 months]
• Complete Response Rate (CR) [Срок оценки: at 30 months]

Критерии участия

Критерии включения

• Previously-untreated patients with de novo diagnosis of peripheral T-cell or NK/T-cell lymphoma:

• T-cell large granular lymphocytic leukaemia;
• Chronic lymphoproliferative disorder of NK cells;
• Aggressive NK-cell leukaemia;
• Adult T-cell leukaemia/lymphoma;
• Extranodal NK/T-cell lymphoma, nasal type;
• Intestinal T-cell lymphoma;
• Hepatosplenic T-cell lymphoma;
• Subcutaneous panniculitis-like T-cell lymphoma;
• Peripheral T-cell lymphoma, not otherwise specified;
• Angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin;
• Anaplastic large cell lymphoma, ALK-positive;
• Anaplastic large cell lymphoma, ALK-negative;
• Breast implant-associated anaplastic large cell lymphoma.
• Age 18 and over;
• Tissue biopsy adequate for diagnosis and classification and available for centralized review;
• Clinical data including baseline information on disease localization and laboratory parameters at staging, features of treatment adopted and assurance of follow-up updating for at least 2 years are requested;
• Written informed consent.

Критерии исключения

• Diagnosis of:

• EBV-positive T-cell and NK-cell lymphoproliferative diseases of childhood
• Mycosis fungoides;
• Sézary syndrome;
• Primary cutaneous CD30-positive T-cell lymphoproliferative disorders;
• Primary cutaneous peripheral T-cell lymphomas, rare subtypes;
• T-cell lymphoblastic lymphoma/leukemia
• T-cell prolymphocitic leukemia
• Age < 18.

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Италия · 3 центра

• IRCCS Istituto Tumori "Giovanni Paolo II" — Bari
• Palermo_La Maddalena — Palermo
• Terni-Santa Maria — Terni

США · 1 центр

• Stanford University — Stanford

Румыния · 1 центр

• Cluj Napoca_Ion Chiricuta Oncology Institute — Cluj-Napoca

Украина · 1 центр

• National Cancer Institute — Kiev

Публикации

• Ansell S. How to select the frontline treatment for a patient with peripheral T-cell lymphoma. Leuk Lymphoma. 2016;57(4):783-8. doi: 10.3109/10428194.2016.1140760. Epub 2016 Feb 5. PMID 26850659
• Vose J, Armitage J, Weisenburger D; International T-Cell Lymphoma Project. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008 Sep 1;26(25):4124-30. doi: 10.1200/JCO.2008.16.4558. Epub 2008 Jul 14. PMID 18626005
• A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project. Blood. 1997 Jun 1;89(11):3909-18. PMID 9166827
• Savage KJ. Peripheral T-cell lymphomas. Blood Rev. 2007 Jul;21(4):201-16. doi: 10.1016/j.blre.2007.03.001. Epub 2007 May 18. PMID 17512649
• Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecul PMID 24632715
• Barbui T, Thiele J, Gisslinger H, Kvasnicka HM, Vannucchi AM, Guglielmelli P, Orazi A, Tefferi A. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. Blood Cancer J. 2018 Feb 9;8(2):15. doi: 10.1038/s41408-018-0054-y. PMID 29426921
• Foss FM, Zinzani PL, Vose JM, Gascoyne RD, Rosen ST, Tobinai K. Peripheral T-cell lymphoma. Blood. 2011 Jun 23;117(25):6756-67. doi: 10.1182/blood-2010-05-231548. Epub 2011 Apr 14. PMID 21493798
• Weisenburger DD, Savage KJ, Harris NL, Gascoyne RD, Jaffe ES, MacLennan KA, Rudiger T, Pileri S, Nakamura S, Nathwani B, Campo E, Berger F, Coiffier B, Kim WS, Holte H, Federico M, Au WY, Tobinai K, Armitage JO, Vose JM; International Peripheral T-cell Lymphoma Project. Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Proj PMID 21270441

Идентификаторы

NCT: NCT03964480 · T-Cell Project 2.0

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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