Long-term results of AIEOP LNH-92 protocol for the treatment of pediatric lymphoblastic lymphoma: a report of the Italian Association of Pediatric Hematology and Oncology.
BACKGROUND
Lymphoblastic lymphoma (LBL) is the second most frequent lymphoma subtype in childhood. It is commonly treated according to therapy strategies for lymphoblastic leukemia.


METHODS
The AIEOP LNH-92 protocol was a modified LSA2-L2 therapy used for both T- and B-cell precursor LBL and included Induction, Consolidation, and Maintenance treatment with a total duration of 11 and 24 months for stages I and II, stages III and IV disease, respectively.


RESULTS
Fifty-five eligible patients were enrolled, 40 males and 15 females, with a median age of 8 years. Complete remission was achieved in 93% of the cases. With a median follow-up of 9 years the event-free survival (EFS) was 69% and overall survival 72%. EFS of localized disease was 100%. The most frequent grades III and IV toxicity was hematologic and hepatic (elevated transaminases) toxicity. No toxic death nor second tumor were observed. Outcome was comparable to most concomitant international protocols for LBL, but inferior to recent trials that included reinduction treatment or a higher intensity therapy for high stage disease.


CONCLUSIONS
AIEOP LNH92 protocol demonstrated similar efficacy compared to contemporary regimens, with limited toxicity. Nevertheless, an intensified treatment is warranted for high stage disease.
Pillon
Marta
M
Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera-Università di Padova, Padova, Italy.
Piglione
Matilde
M
Garaventa
Alberto
A
Conter
Valentino
V
Giuliano
Maria
M
Arcamone
Giampaolo
G
Mura
Rossella
R
Cellini
Monica
M
D'Amore
Emanuele S G
ES
Varotto
Stefania
S
Mussolin
Lara
L
Rosolen
Angelo
A
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D056486:Chemical and Drug Induced Liver Injury; D002648:Child; D002675:Child, Preschool; D005260:Female; D006402:Hematologic Diseases / Q000139:chemically induced; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000401:mortality / Q000628:therapy*; D012074:Remission Induction; D016019:Survival Analysis