title: Detection of prognostic factors in children and adolescents with Burkitt and Diffuse Large B-Cell Lymphoma treated with the AIEOP LNH-97 protocol.
abstract: Burkitt lymphoma (BL) and Diffuse Large B-Cell Lymphoma (DLBCL) account for most cases of non-Hodgkin lymphoma (NHL) in childhood. We report the clinical characteristics, outcome and prognostic factors in children with BL or DLBCL treated according to the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) LNH-97 protocol. Patients aged up to 18 years that were newly diagnosed with BL/DLBCL were included in the study. Therapy consisted of pre-phase followed by 2-6 high-dose chemotherapy courses tailored according to lactate dehydrogenase (LDH) value and disease stage. A total of 442 patients (379 BL, 63 DLBCL) were enrolled between 1997 and 2014, of whom 18 failed to achieve remission, 6 experienced treatment-related death, 2 developed second malignancy and 20 relapsed. At a median follow-up time of 5 years, overall survival was 93% (±1%) and event-free survival was 90% (±1%). LDH value above the median value had an independently negative prognostic value (P < 0·0001). However, in the subgroup of 128 patients in which minimal disseminated disease (MDD) was analysed, MDD-positivity became the only unfavourable prognostic factor for progression-free survival. Tailored chemotherapy could be extremely effective with limited toxicity. Identification of MDD as a hallmark of a higher risk of treatment failure may provide a target population for treatment intensification by anti-CD20.
authors.affiliation: Clinic of Paediatric Haemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy. marta.pillon@unipd.it.
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
authors.affiliation: Clinic of Paediatric Haemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.|Institute of Paediatric Research - Fondazione Città della Speranza, Padova, Italy.
authors.forename: Lara
authors.initials: L
authors.lastname: Mussolin
authors.affiliation: Clinic of Paediatric Haemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Carraro
authors.affiliation: Department of Paediatrics, Ospedale San Gerardo, University of Milano-Bicocca, Fondazione MBBM, Monza, Italy.
authors.forename: Valentino
authors.initials: V
authors.lastname: Conter
authors.affiliation: Medical Department, Paediatric Unit, Azienda Sanitaria Provinciale Ragusa, Ragusa, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.affiliation: Department of Paediatric Haemato-Oncology, IRCCS Ospedale Bambino Gesù, Roma, Italy.
authors.forename: Luciana
authors.initials: L
authors.lastname: Vinti
authors.affiliation: Department of Paediatric Haemato-Oncology, IRCCS I. G. Gaslini, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.affiliation: Department of Paediatric Haemato-Oncology, Regina Margherita Children's Hospital, Torino, Italy.
authors.forename: Matilde
authors.initials: M
authors.lastname: Piglione
authors.affiliation: Department of Paediatric Haemato-Oncology, Santobono-Pausilipon Children's Hospital, Napoli, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Buffardi
authors.affiliation: Department of Paediatrics, Ospedale San Gerardo, University of Milano-Bicocca, Fondazione MBBM, Monza, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sala
authors.affiliation: Department of Paediatric Haemato-Oncology, University of Bari, Bari, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Santoro
authors.affiliation: Department of Paediatric Haemato-Oncology, Azienda Policlinico-OVE, Catania, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Lo Nigro
authors.affiliation: Paediatric Haematology-Oncology, Ospedale Pediatrico Microcitemico, Cagliari, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Mura
authors.affiliation: Department of Paediatric Haematology-Oncology, Azienda Ospedaliero-Universitaria Meyer Children Hospital, Firenze, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Tondo
authors.affiliation: Department of Paediatric Haemato-Oncology, University of Napoli, Napoli, Italy.
authors.forename: Fiorina
authors.initials: F
authors.lastname: Casale
authors.affiliation: Department of Paediatric Haemato-Oncology, ARNAS Ospedali Civico, G Di Cristina, Palermo, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Farruggia
authors.affiliation: Department of Paediatric Haemato-Oncology, Ancona, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Pierani
authors.affiliation: Department of Paediatrics, Paediatric Haematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.
authors.forename: Simone
authors.initials: S
authors.lastname: Cesaro
authors.affiliation: Department of Pathology, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Emanuele S G
authors.initials: ES
authors.lastname: d'Amore
authors.affiliation: Clinic of Paediatric Haemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D015415:Biomarkers; D002051:Burkitt Lymphoma / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D009367:Neoplasm Staging; D018365:Neoplasm, Residual / Q000175:diagnosis; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers
keywords: Associazione Italiana Ematologia Oncologia Pediatrica LNH-97 protocol; B-cell non-Hodgkin lymphoma; childhood; diffuse large B-cell lymphoma; minimal disseminated disease; prognostic factors