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RESEARCH PRODUCT

Prognostic Factors in Childhood Anaplastic Large Cell Lymphoma: Long Term Results of the International ALCL99 Trial

Georg MannAnne UyttebroeckDenise WilliamsWilhelm WoessmannEmanuele S.g. D'amoreChristine Damm-welkElisa CarraroAtsuko NakazawaSuzanne D. TurnerSuzanne D. TurnerAuke BeishuizenRafael Fernández-delgado Fernández-delgado CerdáBirgit BurkhardtMarie-cécile Le DeleyAndishe AttarbaschiMonika CsókaLara MussolinLaurence BrugièresAmos BurkeWolfram KlapperAlfred ReiterKeizo HoribeLaurence LamantKarin MellgrenGrażyna Wróbel

subject

MDD0301 basic medicineOncologyCancer Researchmedicine.medical_specialtyMultivariate analysisIMPACTBONE-MARROWFEATURESCHILDRENlong-term follow-uplcsh:RC254-282ArticleDISEASECLASSIFICATIONChildhood Anaplastic Large Cell Lymphoma03 medical and health sciences0302 clinical medicineInternal medicineADOLESCENTSmedicineVINBLASTINEProgression-free survivalYoung adultPathologicalAnaplastic large-cell lymphomachildhoodScience & Technologybusiness.industryHazard ratioCHEMOTHERAPYlcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogensmedicine.diseaseALCLALCL; MDD; childhood; long-term follow-up030104 developmental biologyOncology030220 oncology & carcinogenesisMinimal Disseminated DiseaseNON-HODGKIN-LYMPHOMAbusinessLife Sciences & Biomedicine

description

With the aim of describing the long-term follow-up and to define the prognostic role of the clinical/pathological/molecular characteristics at diagnosis for childhood, adolescent and young adults affected by anaplastic large cell lymphoma (ALCL), we analyzed 420 patients aged up to 22 years homogeneously treated within the international ALCL99 trial. The 10-year progression free survival (PFS) was 70% and overall survival was 90%, rare late relapses occurred but no secondary malignancies were reported. Among clinical/pathological characteristics, only patients presenting a small cell/lymphohistiocytic (SC/LH) pattern were independently associated with risk of failure (hazard ratio = 2.49). Analysis of minimal disseminated disease (MDD), available for 162 patients, showed that both SC/LH pattern (hazard ratio = 2.4) and MDD positivity (hazard ratio = 2.15) were significantly associated with risk of failure in multivariate analysis. Considering MDD and SC/LH results, patients were separated into three biological/pathological (bp) risk groups: a high-risk group (bpHR) including MDD-positive patients with SC/LH pattern

https://doi.org/10.3390/cancers12102747