Skip Navigation

This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow E-letters: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when E-letters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Seidemann, K.
Right arrow Articles by Reiter, A.
Right arrow Search for Related Content
PubMed
Right arrow Articles by Seidemann, K.
Right arrow Articles by Reiter, A.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Annals of Oncology 11:S141-S145, 2000
© 2000 European Society for Medical Oncology

Symposium Articles

Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trials

K. Seidemann1, G. Henze2, J. D. Beck3, A. Sauerbrey4, J. Kühl5, G. Mann6 and A. Reiter1

1 University children's hospitals of Hannover Germany
2 University children's hospitals of Berlin Germany
3 University children's hospitals of Erlangen Germany
4 University children's hospitals of Jena Germany
5 University children's hospitals of Würzburg Germany
6 St. Anna children's hospital Vienna, Austria

Correspondence to: K. Seidemann, MD Pädiatrische Hämatologie und Onkologie Medizinische Hochschule Hannover Carl Neuberg-Str. 1 30161 Hannover Germany

Background: Lymphoma and leukemia are the commonest malignant diseases in patients with chromosomal breakage syndromes and immunodeficiency (Ataxia teleangiectasia (AT) and Nijmegen breakage syndrome (NBS)). With improved management of infections, malignant disease is more frequently diagnosed and has become one of the commonest causes of death in pediatric AT and NBS.

Patients and methods: In three consecutive multicenter therapy trials for pediatric non-Hodgkin's lymphoma (NHL) (NHL-BFM), 1569 patients with newly diagnosed NHL have been registered between 1986 and 1997. Nine patients with AT (n=5) and NBS (n=4) were identified and analysed.

Results: Median age of patients with AT and NBS at diagnosis of NHL was nine years. NHL-entities differed from non-AT/NBS-patients: diffuse large B-cell lymphomas, n=7 (78%); ALCL, n=1; lymphoblastic T-cell lymphoma, n=1. Cervical nodes, paranasal sinuses and epipharynx were the sites most frequently involved. Stages were: I and II in three patients, III in five and IV in one patient. All patients received polychemotherapy according to tumor-entity and stage, none received radiation. Dose reductions according to individual tolerance concerned mainly ethotrexate, alkylating agents and epipodophyllotoxines. One patient died of toxic complications, two patients relapsed and died, one patient suffered from second malignancy. Five of nine patients are in 1. CCR after a median folluow-up of five years.

Conclusions: Patients with AT and NBS suffer from rare entities of pediatric NHL. Curative treatment is possible and should be attempted. Intensity of therapy should be adjusted to individual risk factors and tolerance. Alkylating agents, epipodophyllotoxines should be omitted, dose of MTX should be limited to 1 g/m2. Further cooperative trials using standardized approaches are required.

AT, ataxia teleangiectasia, Nijmegen-Breakage Syndrome (NBS), non-Hodgkin's lymphoma (NHL)


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?




Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.