RecruitingNCT04411043

Observatory of Prolymphocytic Leukemia T

Prospective and Retrospective Study Evaluating Epidemiological, Clinical, Molecular and Therapeutic Data of Prolymphocytic Leukemia T. A FILO Study.

Sponsor

French Innovative Leukemia Organisation

Enrollment

50 participants

Start Date

Jul 1, 2020

Study Type

OBSERVATIONAL

Conditions

Prolymphocytic Leukemia T-cell Leukemia

Summary

Prolymphocytic leukemia T is a rare disease representing approximately 2% of mature lymphoid leukemias and 20% of prolymphocytic leukemias. It mainly affects the elderly with an aggressive clinical course. It is a hemopathy exhibiting a post thymic T phenotype (Tdt-, CD1a-, CD5 +, CD2 + and CD7 +), generally CD4 + / CD8-, but also CD4 + / CD8 + or CD8 + / CD4-. The main feature of T-PLL is the rearrangement of chromosome 14 involving genes encoding the T cell receptor complex (TCR) subunits, leading to overexpression of the proto-oncogene TCL1. On the molecular level, the study of Prolymphocytic leukemia T shows a substantial mutational activation of the IL2RG-JAK1-JAK3-STAT5B axis. Patients with Prolymphocytic leukemia T have a poor prognosis, due to a poor response to conventional chemotherapy. Treatment with the anti-CD52 monoclonal antibody: alemtuzumab has considerably improved the results, but the responses to treatment are transient; therefore, patients who obtain a response to alemtuzumab treatment are candidates for stem cell allograft (TSS) if they are eligible for this procedure. This combined approach extended the median survival to four years or more. However, new approaches using well-tolerated therapies that target signaling and survival pathways are necessary for most patients who are unable to receive intensive chemotherapy, such as JAK STAT axis inhibitors, anti-AKT, or anti BCL2 . Main objective: Better manage prolymphocytic T leukemias. Secondary objectives: * Molecular characterization of prolymphocytic leukemia T. * Study of the response to treatment, disease-free survival, overall survival. * Impact of prognostic factors on response to treatment, and survival.

Eligibility

Min Age: 18 Years

Plain Language Summary

Simplified for easier understanding

This is a long-term observational registry in France that collects medical information on patients diagnosed with T-cell prolymphocytic leukemia (T-PLL) — a rare and aggressive blood cancer that affects a specific type of white blood cell called T lymphocytes. Because T-PLL is so uncommon, this registry aims to gather enough data to better understand how the disease behaves and how patients respond to different treatments. **You may be eligible if...** - You are 18 years or older (male or female) - You have been diagnosed with T-cell prolymphocytic leukemia (T-PLL), confirmed by your medical team **You may NOT be eligible if...** - You have not signed or are unwilling to sign an informed consent form authorizing use of your data for research purposes Talk to your doctor to see if this trial is right for you.

This summary was AI-generated to explain the trial in plain language. It is not medical advice. Always discuss eligibility with your doctor before enrolling in a clinical trial.

Interested in this trial?

Get notified about updates and connect with the research team.