Diagnosis and management of ALK-positive anaplastic large cell lymphoma in children and adolescents. Issue 1 (March 2023)

• Record Type: Journal Article
• Authors: Rigaud, Charlotte; Knörr, Fabian; Brugières, Laurence; Woessmann, Wilhelm
• Is Part Of: Baillière's best practice and research in clinical haematology. Volume 36:Issue 1(2023)
• Publication Date: 2023-03
• Subjects: Anaplastic large cell lymphoma -- Anaplastic lymphoma kinase -- Children -- Diagnosis -- Therapy; Hematology -- Periodicals; Blood -- Periodicals; Hematologic Diseases -- Periodicals; Electronic journals; 616
• DOI: 10.1016/j.beha.2023.101444
• Languages: English
• ISSNs: 1521-6926

Abstract

Abstract: Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a CD30-positive T cell lymphoma characterized by signalling from constitutively activated ALK fusion proteins. Most children and adolescents present in advanced stages, often with extranodal disease and B symptoms. The current front-line therapy standard of six cycles polychemotherapy reaches an event-free survival of 70%. The strongest independent prognostic factors are minimal disseminated disease and early minimal residual disease. At relapse, ALK-inhibitors, Brentuximab Vedotin, Vinblastine, or second line chemotherapy are effective re-inductions. Survival at relapse exceeds 60–70% with consolidation according to the time of relapse (Vinblastine monotherapy or allogeneic hematopoietic stem cell transplantation) so that the overall survival reaches 95%. It needs to be shown whether check-point inhibitors or long-term ALK-inhibition may substitute for transplantation. The future necessitates international cooperative trials testing whether a shift of paradigm to a chemotherapy-free regimen can cure ALK-positive ALCL.