Profound and sustained response with next-generation ALK inhibitors in patients with relapsed or progressive ALK-positive anaplastic large cell lymphoma with central nervous system involvement

Profound and sustained response with next-generation ALK inhibitors in patients with relapsed or progressive ALK-positive anaplastic large cell lymphoma with central nervous system involvement Anaplastic large cell lymphoma (ALCL) is a rare disease accounting for less than 15% of all non-Hodgkin lymphomas in childhood. 5] By contrast, next-generation ALK inhibitors, which have been developed for lung cancer and brain metastases, have good CNS penetration and could therefore be of great interest for treating patients with ALK-positive ALCL and CNS involvement. We prospectively collected data on all French patients <22 years old treated between 2017 and 2020 with next-generation ALK inhibitors for a CNS relapse/progression of ALK-positive ALCL. Ten patients, suffering from 11 CNS relapses/progression, were identified. Data for each individual and details of each CNS relapse/progression are available in Tables One patient only had CNS involvement at diagnosis. Of note, all patients underwent a diagnostic cerebrospinal fluid evaluation as routine staging. CNS imaging was only performed in cases of clinical suspicion. Three patients had a leukemic presentation or bone marrow involvement at diagnosis and six had no particular clinical risk factor for CNS evolution (Table Of note, at frontline therapy, all patients at diagnosis, except for one, were at high risk of relapse or progression with minimal disseminated disease and minimal residual disease after one course of treatment. Five patients experienced disease progression while on frontline therapy. For the five others who achieved complete remission and completed the frontline therapy schedule, the time from end of treatment to the first relapse ranged from 0.6 to 2.1 months.