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Blood, 1 February 2008, Vol. 111, No. 3, pp. 1560-1566.
Prepublished online as a Blood First Edition Paper on October 23, 2007; DOI 10.1182/blood-2007-07-100958.
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Submitted July 24, 2007
Accepted October 10, 2007
Prognostic factors in childhood anaplastic large cell lymphoma : results of a large European Intergroup Study
Marie-Cecile Le Deley*, Alfred Reiter, Denise Williams, Georges Delsol, Ilske Oschlies, Keith McCarthy, Martin Zimmermann, and Laurence Brugieres
Biostatistics and Epidemiology Unit, Institut Gustave-Roussy, Villejuif, France
Department of Pediatric Hematology and Oncology, NHL-BFM Study Centre, Justus Liebig University, Giessen, Germany
Department of Pediatrics, Addenbrookes Hospital, Cambridge, United Kingdom
Department of Pathology, INSERM, U563, Centre de Physiopathologie de Toulouse Purpan, Universite Toulouse III Paul-Sabatier, Toulouse, France
Hematophathology Section and Lymph Node Registry, University of Kiel, Kiel, Germany
Department of Pathology, Gloucester Hospitals NHS Foundation Trust, Gloucester, United Kingdom
Department of Pediatric Hematology and Oncology, Medizinische Hochschule Hannover, Hannover, Gibraltar
Department of Pediatrics, Institut Gustave Roussy, Villejuif, France
* Corresponding author; email: le_deley{at}igr.fr.
To study prognostic factors of progression/relapse, data concerning 225 children enrolled between 1987 and 1997 in BFM, SFOP and UKCCSG prospective studies for the treatment of anaplastic large cell lymphoma were merged. Median follow-up was 9.3 years. Five-year overall survival and event-free survival (EFS) of the whole population was 81% [95%CI, 76-86%] and 69% [63-74%] respectively. B symptoms, mediastinal involvement, skin lesions, visceral involvement, St Jude stage 3-4, Ann Arbor stage 3-4 and elevated LDH increased the risk of progression/relapse in the univariate analysis. In the multivariate analysis, three factors remained significant: mediastinal involvement (RR=2.1 [1.2-3.5]), visceral involvement defined as lung, liver or spleen involvement (RR=2.1 [1.3-3.6]) and skin lesions (RR=1.9 [1.1-3.2]). Five-year PFS of the 81 patients with none of these risk factors was 89% [82-96%], contrasting with a 5-y PFS of 61% [53-69%] in the 144 patients with  1 risk factor (RR=4.4 [2.2-8.9, p<10-5). In conclusion, three factors associated with an increased risk of failure in childhood ALCL have been defined: mediastinal involvement, visceral involvement and skin lesions.
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