Non‐Hodgkin lymphoma (NHL) in children with Nijmegen Breakage syndrome (NBS)
- 5 December 2008
- journal article
- research article
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 52 (2), 186-190
- https://doi.org/10.1002/pbc.21789
Abstract
Background Due to small number of patients with Nijmegen Breakage Syndrome (NBS) and Non‐Hodgkin lymphoma (NHL) experience in their treatment is limited. Procedure Since 1996, 17 patients with a median age of 9.5 years who had NBS, were treated for NHL. NHL type, stage, chemotherapy, dose modifications, chemotherapy delays, response to chemotherapy, toxicity, outcome and correlation of drug reduction with response to treatment and outcome were analyzed. Results Nine patients had TNHL, eight BNHL. TNHL patients received BFM and BNHL LMB type protocols. Doses of cytostatics were reduced in the first chemotherapy courses. Further modifications depended on severity of complications. None of the patients complied with timing of chemotherapy. Complete remissions after induction were achieved in 8 of 9 TNHL and 3 out 8 of BNHL patients. All patients experienced grade 4 toxicities. Two patients died from complications. Six of 17 patients are alive. All received more than 80% of recommended doses of chemotherapy. No differences in the type, number of responses or grade 3 and 4 toxicities between patients receiving less or more than 80% of drug doses were observed. Treatment related deaths concerned patients who received less than 80% of drug doses. Conclusions Patients with NBS develop both T and B cell lymphomas. Treatment outcome is poor and might be improved by administering over 80% of drug doses. Although toxicity often depends upon drug doses, our patients experienced equal grade 3 and 4 toxicities whether they received more or less than 80% of the chemotherapeutic agents. Pediatr Blood Cancer 2009;52:186–190.This publication has 11 references indexed in Scilit:
- A Prospective Study on the Epidemiology of Febrile Episodes during Chemotherapy-Induced Neutropenia in Children with Cancer or after Hemopoietic Stem Cell TransplantationClinical Infectious Diseases, 2007
- Cancer incidence in Nijmegen breakage syndrome is modulated by the amount of a variant NBS protein.Carcinogenesis: Integrative Cancer Research, 2007
- Treatment of mature B-ALL and high grade B-NHL in childrenBest Practice & Research Clinical Haematology, 2002
- Anthracyclines in Nijmegen breakage syndromeMedical and Pediatric Oncology, 2002
- Chromosome breakage syndromes and cancerAmerican Journal of Medical Genetics, 2002
- The Societe Francaise d'Oncologie Pediatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B-cell lymphomas and L3 leukemiaBlood, 2001
- Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trialsAnnals of Oncology, 2000
- Therapy for non-Hodgkin lymphoma in children with primary immunodeficiency: Analysis of 19 patients from the BFM trialsMedical and Pediatric Oncology, 1999
- Eleven polish patients with microcephaly, immunodeficiency, and chromosomal instability: The Nijmegen breakage syndromeAmerican Journal of Medical Genetics, 1995
- Non-Hodgkin's lymphomas of childhood and adolescence: results of a treatment stratified for biologic subtypes and stage--a report of the Berlin-Frankfurt-Münster Group.Journal of Clinical Oncology, 1995