Open Access
Prognostic factors in paediatric anaplastic large cell lymphoma: role of ALK
Christine Damm-Welk1,3,Marta Pillon2,Wilhelm Woessmann1,3,*,Lara Mussolin2,3
Dept. of Paediatric Haematology and Oncology and NHL-BFM Study Centre, Justus-Liebig-University, Feulgenstrasse 12, 35392 Giessen, Germany
Istituto di Ricerca Pediatrico Fondazione Città della Speranza, Corso Stati Uniti 4, 35127 Padova, Italy and Department of Paediatric Haematology and Oncology, University of Padova, Via Giustiniani 3, 35128 Padova, Italy
European Research Initiative on ALK-related malignancies (ERIA)
DOI: 10.2741/S434 Volume 7 Issue 2, pp.205-216
Published: 01 June 2015
(This article belongs to the Special Issue ALK: 20 years of discoveries)
*Corresponding Author(s):  
Wilhelm Woessmann

Event-free survival of children and adolescents with ALK-positive anaplastic large cell lymphoma (ALCL) reaches 65-75% with current chemotherapy regimen. Risk stratification of children with ALCL was, until now, based on clinical parameters. More recently, pathological and biological risk factors have been described in trials applying BFM-type chemotherapy. Histological subtypes containing small-cell or lymphohistiocytic components indicate a high risk of failure. Minimal disseminated disease (MDD) detected by qualitative RT-PCR for NPM-ALK in bone marrow or blood is associated with a relapse risk of 50%. Quantification of MDD and persistent minimal residual disease (MRD) characterize very high risk patients. Serum ALK-autoantibody titres inversely correlate with relapse risk. The combination of MDD and ALK-antibody titre separates both low and very high risk patients from those with standard risk. In relapse, the time of relapse/progression, central nervous system and bone marrow involvement are major risk factors. In conclusion, MDD, MRD, ALK-antibody titres and histological subtype are strong biological risk factors in childhood ALCL. The combination of MDD and ALK-antibody titre may serve for patient stratification in upcoming clinical trials.

Key words

Anaplastic Large Cell Lymphoma, ALCL, Children, ALK, Risk factor, Minimal disseminated disease, MRD, ALK antibody, Histological subtype, Review

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Christine Damm-Welk, Marta Pillon, Wilhelm Woessmann, Lara Mussolin. Prognostic factors in paediatric anaplastic large cell lymphoma: role of ALK. Frontiers in Bioscience-Scholar. 2015. 7(2); 205-216.