T-PLL: Observatory of Prolymphocytic Leukemia T
Study Details
Study Description
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:
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Molecular characterization of prolymphocytic leukemia T.
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Study of the response to treatment, disease-free survival, overall survival.
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Impact of prognostic factors on response to treatment, and survival.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
|
Study Design
Outcome Measures
Primary Outcome Measures
- 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
- 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)
- Flow cytometry data of bone marrow and blood cells [At day 0 and at relapse, an average of 3 years]
Positive or negative immunophenotyping
- karyotype of tumor cells [At day 0 and at relapse, an average of 3 years]
karyotipic formula
Eligibility Criteria
Criteria
Inclusion Criteria:
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Man or woman aged 18 or over
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Patient with prolymphocytic T leukemia
Exclusion Criteria:
- Absence of signature of informed consent
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | Chd Le Mans | Le Mans | France | 72000 |
Sponsors and Collaborators
- French Innovative Leukemia Organisation
- University Hospital, Lille
Investigators
- Principal Investigator: Kamel LARIBI, Dr, French Innovative Leukemia Organisation
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- T-PLL