title: Promising therapy results for lymphoid malignancies in children with chromosomal breakage syndromes (Ataxia teleangiectasia or Nijmegen-breakage syndrome): a retrospective survey.
abstract: Children with chromosomal instability syndromes have an increased risk of developing lymphoma and leukaemia. The treatment of these malignancies is hampered by therapy-associated toxicity and infectious complications. This retrospective analysis evaluated the therapy outcome of 38 children with Ataxia teleangiectasia or Nijmegen-breakage syndrome with acute lymphoblastic leukaemia (ALL, n = 9), Non-Hodgkin lymphoma (NHL, n = 28) and Hodgkin lymphoma (HL, n = 1). All patients with NHL or ALL were treated in accordance to Berlin-Frankfurt-Münster (BFM)- or Co-operative study group for childhood ALL (CoALL)-oriented chemotherapy schedules. 22 patients received significantly reduced-intensity chemotherapy. After a median follow-up of 3·7 years the 10-year overall survival was 58%. Dosage-reduction of chemotherapeutic drugs seemed to have no disadvantages and reduced toxic side effects. On the other hand, reduced-intensity chemotherapy did not prevent second malignancies, which occurred in ten patients with a 10-year incidence of 25%. After individual treatment approaches three of these patients with second malignancies were in complete clinical remission for more than 5 years. We conclude that BFM- or CoALL-oriented chemotherapy is effective and can be administered in children with AT or NBS. Moreover, we show that even second lymphoid malignancies can successfully be treated in these patients.
authors.affiliation: Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, Heinrich Heine University, Düsseldorf, Germany.
authors.forename: Klaus
authors.initials: K
authors.lastname: Bienemann
authors.forename: Birgit
authors.initials: B
authors.lastname: Burkhardt
authors.forename: Simon
authors.initials: S
authors.lastname: Modlich
authors.forename: Ulrike
authors.initials: U
authors.lastname: Meyer
authors.forename: Anja
authors.initials: A
authors.lastname: Möricke
authors.forename: Kirsten
authors.initials: K
authors.lastname: Bienemann
authors.forename: Christine
authors.initials: C
authors.lastname: Mauz-Körholz
authors.forename: Gabriele
authors.initials: G
authors.lastname: Escherich
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Dieter
authors.initials: D
authors.lastname: Körholz
authors.forename: Gritta
authors.initials: G
authors.lastname: Janka-Schaub
authors.forename: Martin
authors.initials: M
authors.lastname: Schrappe
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Arndt
authors.initials: A
authors.lastname: Borkhardt
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D008297:Male; D049932:Nijmegen Breakage Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology; D012189:Retrospective Studies