Informations générales (source: ClinicalTrials.gov)
Prospective and Retrospective Study Evaluating Epidemiological, Clinical, Molecular and Therapeutic Data of Prolymphocytic Leukemia T (T-PLL)
Observational [Patient Registry]
French Innovative Leukemia Organisation (Voir sur ClinicalTrials)
juillet 2020
juin 2025
29 juin 2024
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.
Etablissements
| Les établissements d'Île-de-France ayant mis à jour leurs données Origine et niveau de fiabilité des données | |||||
|---|---|---|---|---|---|
| HOPITAL NOVO | GONZALEZ | 04/07/2024 11:05:06 | Contacter | ||
| Les établissements sans correspondance certaine dans le répertoire FINESS dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données | |||||
| Chd Le Mans - 72000 - Le Mans - France | Kamel LARIBI, MD | Contact (sur clinicalTrials) | |||
Critères
Tous
Inclusion Criteria:
- Man or woman aged 18 or over
- Patient with prolymphocytic T leukemia
- Man or woman aged 18 or over
- Patient with prolymphocytic T leukemia
- Absence of signature of informed consent