IMR Press / FBS / Volume 7 / Issue 2 / DOI: 10.2741/S434

Frontiers in Bioscience-Scholar (FBS) is published by IMR Press from Volume 13 Issue 1 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.

Prognostic factors in paediatric anaplastic large cell lymphoma: role of ALK
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1 Dept. of Paediatric Haematology and Oncology and NHL-BFM Study Centre, Justus-Liebig-University, Feulgenstrasse 12, 35392 Giessen, Germany
2 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
3 European Research Initiative on ALK-related malignancies (ERIA)

*Author to whom correspondence should be addressed.


Front. Biosci. (Schol Ed) 2015, 7(2), 205–216;
Published: 1 June 2015

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.

Anaplastic Large Cell Lymphoma
Risk factor
Minimal disseminated disease
ALK antibody
Histological subtype
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