Clinical trial NCT04411043: Observatory of Prolymphocytic Leukemia T
Official title: Prospective and Retrospective Study Evaluating Epidemiological, Clinical, Molecular and Therapeutic Data of Prolymphocytic Leukemia T
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Prolymphocytic Leukemia
T-cell Leukemia
Lead sponsor: French Innovative Leukemia Organisation
Sponsor class: OTHER
Collaborators: name: University Hospital, Lille
First posted: 2020-06-02
Enrollment: 50
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patient with prolymphocytic T leukemia

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.


Eligibility criteria: Inclusion Criteria:

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

Exclusion Criteria:

* Absence of signature of informed consent

Primary outcomes:
measure: Clinical characteristics of prolymphocytic leukemia T
timeFrame: from day 0 through study completion, an average of 3 years
measure: Biological characteristics of prolymphocytic leukemia T
timeFrame: At day 0 and at relapse, an average of 3 years
measure: Flow cytometry data of bone marrow and blood cells
timeFrame: At day 0 and at relapse, an average of 3 years
measure: karyotype of tumor cells
timeFrame: At day 0 and at relapse, an average of 3 years
Secondary outcomes:
Other outcomes:
Locations:
city: Le Mans
country: France
facility: Chd Le Mans
Study officials:
affiliation: French Innovative Leukemia Organisation
name: Kamel LARIBI, Dr
role: PRINCIPAL_INVESTIGATOR
References: