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Blood, Vol. 92 No. 10 (November 15), 1998:
pp. 3591-3598
CD30+ Anaplastic Large-Cell Lymphoma in Children:
Analysis of 82 Patients Enrolled in Two Consecutive Studies of
the French Society of Pediatric Oncology
L. Brugières,
M.C. Le Deley,
H. Pacquement,
Z. Meguerian-Bedoyan,
M.J. Terrier-Lacombe,
A. Robert,
C. Pondarré,
G. Leverger,
C. Devalck,
C. Rodary,
G. Delsol, and
O. Hartmann
From the Departments of Pediatric Oncology, Statistics, and
Pathology, Institut Gustave-Roussy, Villejuif, France; the Department
of Pediatric Oncology, Institut Curie, Paris, France; the Departments
of Pathology and of Pediatric Hematology and Oncology, CHU Purpan,
Toulouse, France; the Department of Pediatric Hematology, Hopital
Debrousse, Lyon, France; the Department of Pediatric Onco-Hematology,
Hopital Trousseau, Paris, France; and the Department of
Onco-Hematology, Hopital des Enfants Reine Fabiola, Bruxelles, Belgium.
The purpose of this study was (1) to investigate the efficacy of
chemotherapy regimens designed by the French Society of Pediatric Oncology for childhood anaplastic large-cell lymphoma (ALCL) and (2) to
identify prognostic factors in these children. Eighty-two children with
newly diagnosed ALCL were enrolled in two consecutive studies, HM89 and
HM91. The diagnosis of ALCL was based on immuno-morphological features
and all the cases but 2 were investigated using ALK1 antibody directed
to the NPM/ALK protein associated with the 2;5 translocation. Treatment
consisted of 2 courses of COPADM (methotrexate, cyclophosphamide,
doxorubicin, vincristine, and prednisone) and a maintenance treatment
of 5 to 7 months. Seventy-eight patients (95%) achieved a complete
remission and 21 relapsed. The probability of survival and event-free
survival at 3 years was of 83% (72% to 90%) and 66% (54% to 76%),
respectively, with a median follow-up of 49 months. In multivariate
analysis, visceral involvement, mediastinal involvement, and
lacticodeshydrogenase (LDH) level  800 UI/L were shown
to be predictive of a higher risk of failure. In conclusion, this type
of regimen demonstrated efficacy in childhood ALCL. However,
therapeutic results have to be improved for children with adverse
prognostic parameters such as visceral or mediastinal involvement or a
high LDH level.
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