Observatory of Prolymphocytic Leukemia T

Recruiting

• Conditions: T-cell Leukemia; Prolymphocytic Leukemia

• Registration Number: NCT04411043
• Lead Sponsor: French Innovative Leukemia Organisation

Brief 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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2020-05-13
• Study First Submitted QC: 2020-05-27
• Study First Posted: 2020-06-02
• Study Start: 2020-07-01
• Status Verified: 2024-10
• Last Update Submitted: 2024-10-25
• Last Update Posted: 2024-10-29
• Primary Completion: 2025-06-30
• Study Completion: 2025-06-30

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 50

Inclusion Criteria

• Man or woman aged 18 or over
• Patient with prolymphocytic T leukemia

Exclusion Criteria

• Absence of signature of informed consent

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
karyotype of tumor cells	At day 0 and at relapse, an average of 3 years	karyotipic formula
Biological characteristics of prolymphocytic leukemia T	At day 0 and at relapse, an average of 3 years	Blood count : Hemoglobin, Leukocytes, Lymphocytes, Platelets, Eosinophils (giga / liters)
Clinical characteristics of prolymphocytic leukemia T	from day 0 through study completion, an average of 3 years	pathology description at diagnosis and its evolution over time
Flow cytometry data of bone marrow and blood cells	At day 0 and at relapse, an average of 3 years	Positive or negative immunophenotyping

Trial Locations

Locations (1)

Chd Le Mans; 🇫🇷 Le Mans, France