ALK-positive anaplastic large cell (anaplastic large-cell lymphoma, ALCL) is a rare adult disease for which almost all published studies are retrospective. The overall survival (OS) at 5 years varies from 70 to 87%. A recent international pooled analysis showed that the International Prognostic Index (IPI) had good predictive value for the PFS and the OS.
In children, new prognostic factors have been identified: the titre of circulating anti-ALK antibodies (inverse correlation between the antibody titre and the prognosis), the detection of a NPM-ALK transcript in the blood at diagnosis (minimal disseminated disease, MDD); correlation between MDD positivity and pejorative prognosis) and its minimal residual disease (MRD), and the histological subtype (common versus small cells / lymphohistiocytic).
The predictive value of the oncogenic profile of ALK-positive ALCL and circulating tumor DNA will be studied in this study.
* At diagnosis (before starting treatment):
* Cycle 2 Day 1 (before starting cycle 2 treatment):
* At the end of treatment evaluation:
40 patients treated with 6 cycles of CHOEP every 3 weeks and 1 lumbar puncture at diagnosis with intrathecal prophylaxis with methotrexate will be included prospectively.
During a 2-year period, relapses / progressions and patient deaths will be monitored for the calculation of the incidence of relapse / progression, progression free survival (PFS) and overall survival (OS).
