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

Status: Recruiting
Location: See location...
Intervention Type: Behavioral
Study Type: Observational
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
Participation Requirements
Sex: All
Minimum Age: 18
Healthy Volunteers: f
View:

• Man or woman aged 18 or over

• Patient with prolymphocytic T leukemia

Locations
Other Locations
France
Chd Le Mans
RECRUITING
Le Mans
Contact Information
Primary
Charles HERBAUX, Dr
Charles.herbaux@chru-lille.fr
3 20 44 57 13
Backup
Alexandra FAYAULT
a.fayault@filo-leucemie.org
Time Frame
Start Date: 2020-07-01
Estimated Completion Date: 2025-06-30
Participants
Target number of participants: 50
Sponsors
Leads: French Innovative Leukemia Organisation
Collaborators: University Hospital, Lille

This content was sourced from clinicaltrials.gov