Anaplastic large cell lymphoma treated with a leukemia-like therapy: report of the Italian Association of Pediatric Hematology and Oncology (AIEOP) LNH-92 protocol.
BACKGROUND
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Rosolen
Angelo
A
Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera-Università di Padova, Padova, Italy. angelo-rosolen@unipd.it
Pillon
Marta
M
Garaventa
Alberto
A
Burnelli
Roberta
R
d'Amore
Emanuele S
ES
Giuliano
Maria
M
Comis
Margherita
M
Cesaro
Simone
S
Tettoni
Katia
K
Moleti
Maria Luisa
ML
Tamaro
Paolo
P
Visintin
Gianluca
G
Zanesco
Luigi
L
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D003630:Daunorubicin / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007938:Leukemia / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D009367:Neoplasm Staging; D011241:Prednisone / Q000627:therapeutic use; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D003520:Cyclophosphamide; D011241:Prednisone; D008727:Methotrexate; D003630:Daunorubicin