Anaplastic large cell lymphoma treated with a leukemia-like therapy : Report of the Italian association of pediatric hematology and oncology (AIEOP) LNH-92 protocol

• Published in: Cancer 2005-11, Vol.104 (10), p.2133-2140
• Main Authors: ROSOLEN, Angelo, PILLON, Marta, TAMARO, Paolo, VISINTIN, Gianluca, ZANESCO, Luigi, GARAVENTA, Alberto, BURNELLI, Roberta, D'AMORE, Emanuele S, GIULIANO, Maria, COMIS, Margherita, CESARO, Simone, TETTONI, Katia, MOLETI, Maria Luisa
• Format: Article
• Language: English
• Subjects: Adolescent; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Cyclophosphamide - therapeutic use; Daunorubicin - therapeutic use; Disease-Free Survival; Female; Hematologic and hematopoietic diseases; Humans; Immunohistochemistry; Leukemia - therapy; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - mortality; Lymphoma, Large B-Cell, Diffuse - pathology; Male; Medical sciences; Methotrexate - therapeutic use; Neoplasm Staging; Prednisone - therapeutic use; Survival Analysis; Treatment Outcome; Tumors; Vincristine - therapeutic use
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/cncr.21438

Childhood anaplastic large cell lymphoma (ALCL) is a well defined entity with a rather poor prognosis. Different approaches have been adopted in the treatment of ALCL in various cooperative trials, including short high-dose intensive therapy and leukemia-like protocols. In the early 1990s, the Italian Association of Pediatric Hematology and Oncology (AIEOP) initiated a multicenter trial for the treatment of ALCL based on a modified LSA2-L2 protocol. Thirty-four consecutive eligible children with newly diagnosed ALCL were enrolled in the AIEOP LNH-92 protocol. Treatment was comprised of an induction of remission phase, followed by consolidation and maintenance for a total duration of 24 months, independently of disease stage. Thirty of 34 patients (88%) achieved complete disease remission and 8 patients experienced disease recurrence. With a median follow-up of 8.4 years, the probabilities of survival and event-free survival were 85% (range, 79-91%) and 65% (range, 57-73%), respectively. Therapy was well tolerated and hematologic toxicity was the most frequent toxicity. The leukemia-like protocol AIEOP LNH-92 was found to be an effective treatment for childhood ALCL. Its long duration may be beneficial to specific patient subgroups, but optimal treatment duration in ALCL remains to be elucidated.