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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.
NEOPLASIA Prognostic factors in childhood anaplastic large cell lymphoma: results of a large European intergroup study1 Biostatistics and Epidemiology Unit, Institut Gustave-Roussy, Villejuif, France; 2 University Paris-Sud, Le Kremlin-Bicêtre, France; 3 Department of Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany; 4 Department of Pediatrics, Addenbrookes Hospital, Cambridge; United Kingdom; 5 Inserm, U563, Centre de Physiopathologie de Toulouse Purpan; Université Toulouse III Paul-Sabatier; Toulouse, France; 6 Department of Pathology, Centre Hospitalo-Universitaire Toulouse, Hôpital Purpan, Toulouse, France; 7 Department of Pathology, Hematopathology Section and Lymph Node Registry, University of Kiel, Kiel, Germany; 8 Department of Pathology, Gloucester Hospitals National Health Service Foundation Trust, Gloucester, United Kingdom; 9 Department of Pediatric Hematology and Oncology, Medizinische Hochschule Hannover, Hannover, Germany; and 10 Department of Pediatrics, Institut Gustave-Roussy, Villejuif, France To study prognostic factors of progression/relapse, data concerning 225 children enrolled between 1987 and 1997 in Berlin-Frankfurt-Münster, Société Française d'Oncologie Pédiatrique and United Kingdom Children's Cancer Study Group prospective studies for the treatment of anaplastic large cell lymphoma (ALCL) were merged. Median follow-up was 9.3 years. Five-year overall survival and event-free survival of the whole population was 81% (95% confidence interval, 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 lactate dehydrogenase increased the risk of progression/relapse in the univariate analysis. In the multivariate analysis, 3 factors remained significant: mediastinal involvement (relative risk [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 progression-free survival (PFS) of the 81 patients with none of these risk factors was 89% [82%-96%], contrasting with a 5-year PFS of 61% [53%-69%] in the 144 patients with at least 1 risk factor (RR = 4.4 [2.2-8.9; P < .001). In conclusion, 3 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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