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

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.

Inclusion criteria

• 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.

Exclusion criteria

• 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.