Promising therapy results for lymphoid malignancies in children with chromosomal breakage syndromes (Ataxia teleangiectasia or Nijmegen-breakage syndrome): a retrospective survey.
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Promising therapy results for lymphoid malignancies in children with chromosomal breakage syndromes (Ataxia teleangiectasia or Nijmegen-breakage syndrome): a retrospective survey. / Bienemann, Klaus; Burkhardt, Birgit; Modlich, Simon; Meyer, Ulrike; Möricke, Anja; Bienemann, Kirsten; Mauz-Körholz, Christine; Escherich, Gabriele; Zimmermann, Martin; Körholz, Dieter; Janka-Schaub, Gritta; Schrappe, Martin; Reiter, Alfred; Borkhardt, Arndt.
in: BRIT J HAEMATOL, Jahrgang 155, Nr. 4, 4, 2011, S. 468-476.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Promising therapy results for lymphoid malignancies in children with chromosomal breakage syndromes (Ataxia teleangiectasia or Nijmegen-breakage syndrome): a retrospective survey.
AU - Bienemann, Klaus
AU - Burkhardt, Birgit
AU - Modlich, Simon
AU - Meyer, Ulrike
AU - Möricke, Anja
AU - Bienemann, Kirsten
AU - Mauz-Körholz, Christine
AU - Escherich, Gabriele
AU - Zimmermann, Martin
AU - Körholz, Dieter
AU - Janka-Schaub, Gritta
AU - Schrappe, Martin
AU - Reiter, Alfred
AU - Borkhardt, Arndt
PY - 2011
Y1 - 2011
N2 - 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.
AB - 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.
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Child
KW - Disease-Free Survival
KW - Child, Preschool
KW - Retrospective Studies
KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
KW - Lymphoma, Non-Hodgkin/drug therapy/genetics/pathology
KW - Nijmegen Breakage Syndrome/complications/genetics/pathology
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Child
KW - Disease-Free Survival
KW - Child, Preschool
KW - Retrospective Studies
KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
KW - Lymphoma, Non-Hodgkin/drug therapy/genetics/pathology
KW - Nijmegen Breakage Syndrome/complications/genetics/pathology
M3 - SCORING: Journal article
VL - 155
SP - 468
EP - 476
JO - BRIT J HAEMATOL
JF - BRIT J HAEMATOL
SN - 0007-1048
IS - 4
M1 - 4
ER -
