Neuro-meningeal relapse in anaplastic large-cell lymphoma: incidence, risk factors and prognosis - a report from the European intergroup for childhood non-Hodgkin lymphoma

Br J Haematol. 2021 Mar;192(6):1039-1048. doi: 10.1111/bjh.16755. Epub 2020 Jul 9.


Relapses involving the central nervous system (CNS) are rare in children and adolescents with ALK+ anaplastic large cell lymphoma (ALCL) treated with regimens including CNS prophylaxis. Early identification of patients at high-risk for CNS relapse would enable stratification and better adaptation of initial treatment especially in the light of the upcoming targeted therapies with limited CNS penetration. We analyzed clinical and histological data of all ALK+ALCL patients with CNS relapse registered in ALCL99-database with the aim to describe risk factors and outcome. Characteristics of patients with no relapse, relapse without CNS involvement and CNS relapse were compared. At a median follow-up of 8 years (0.05-18 years), a CNS involvement was reported at first or subsequent relapse in 26/618 patients. Median interval between initial diagnosis and first CNS relapse was 8 months (IQR 5.55-10.61/range 1.31-130.69). The 5-year cumulative risk of CNS relapse was 4% (95% CI 2.9-5.5). Bone marrow involvement, peripheral blasts and CNS involvement at diagnosis were more frequent in patients with CNS relapse than in patients with no relapse or with relapse with no CNS involvement. The treatment of CNS relapse was heterogeneous. The median survival after CNS relapse was 23.7 months. Eleven patients were alive at last follow-up. Three-year overall survival after CNS relapse was 48.70% (95% CI 30.52-67.23).

Keywords: ALK; CNS prophylaxis; anaplastic large cell lymphoma; neuro-meningeal relapse; risk factors.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Randomized Controlled Trial

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Databases, Factual
  • Disease-Free Survival
  • Europe / epidemiology
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Infant
  • Lymphoma, Large-Cell, Anaplastic / mortality*
  • Lymphoma, Large-Cell, Anaplastic / therapy
  • Male
  • Meningeal Neoplasms / mortality*
  • Meningeal Neoplasms / therapy
  • Recurrence
  • Risk Factors
  • Survival Rate