title: Detection and role of minimal disseminated disease in children with lymphoblastic lymphoma: The AIEOP experience.
abstract: BACKGROUND
  The use of intensive chemotherapy regimens in children with lymphoblastic lymphoma (LBL) has significantly improved outcome, but the salvage rate for these patients is still poor. The aim of this study was to evaluate the prognostic value of minimal disseminated disease (MDD), studied by multiparametric flow cytometry (MFC), in pediatric patients with T- and B-lineage LBL.
  
  
  METHODS
  We examined bone marrow (BM) and peripheral blood (PB) samples from a series of 65 children affected by T- (52) and B-lineage (13) LBL using an MFC method; 10 of them were also analyzed for clonality of T-cell receptor gene rearrangements.
  
  
  RESULTS
  MDD was detected in 49% (32/65) of BM samples, whereas only 21% (14/65) were positive at standard morphological evaluation. Findings from MFC analyses of paired BM and PB samples were highly concordant. We analyzed the prognostic significance of MDD results detected at diagnosis in morphologically negative patients, as almost all relapsed cases (10/11) did not have any morphological involvement of BM at diagnosis. Using an MDD cut-off level of 3% by FCM (75th percentile), 5-year event-free survival (EFS) was 60% (SE ± 22) for patients with MDD >3% LBL cells versus 83% (SE ± 6) for the remaining patients (P = 0.04). No statistically significant difference in EFS was observed between LBL patients considering all the other clinical characteristics.
  
  
  CONCLUSIONS
  Our data demonstrated that MDD studied at diagnosis by MFC could represent a useful prognostic tool in childhood LBL and further application for better stratification is warranted.
authors.affiliation: Istituto di Ricerca Pediatrico-Fondazione Città della Speranza, Padua, Italy.|Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Lara
authors.initials: L
authors.lastname: Mussolin
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Buldini
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Lovisa
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Carraro
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Disarò
authors.affiliation: Center of Pediatric Hematology Oncology, Azienda Policlinico, Catania, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Lo Nigro
authors.affiliation: Department of Pathology, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Emanuele S G
authors.initials: ES
authors.lastname: d'Amore
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padua, Padua, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
mesh_terms: D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D001854:Bone Marrow Cells / Q000378:metabolism / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D008297:Male; D018365:Neoplasm, Residual; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000378:metabolism / Q000401:mortality / Q000473:pathology; D011947:Receptors, Antigen, B-Cell / Q000378:metabolism; D011948:Receptors, Antigen, T-Cell / Q000378:metabolism; D015996:Survival Rate; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology
chemical_list: D011947:Receptors, Antigen, B-Cell; D011948:Receptors, Antigen, T-Cell
keywords: childhood; flow cytometry; lymphoblastic lymphoma; minimal disseminated disease