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  • 1
    ISSN: 1569-8041
    Keywords: AT ; ataxia teleangiectasia ; Nijmegen-Breakage Syndrome (NBS) ; non-Hodgkin's lymphoma (NHL)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:Lymphoma and leukemia are the commonest malignantdiseases in patients with chromosomal breakage syndromes and immunodeficiency(Ataxia teleangiectasia (AT) and Nijmegen breakage syndrome (NBS)). Withimproved management of infections, malignant disease is more frequentlydiagnosed and has become one of the commonest causes of death in pediatric ATand NBS. Patients and methods:In three consecutive multicenter therapytrials for pediatric non-Hodgkin's lymphoma (NHL) (NHL-BFM), 1569 patientswith newly diagnosed NHL have been registered between 1986 and 1997. Ninepatients with AT (n = 5) and NBS (n = 4) were identified andanalysed. Results:Median age of patients with AT and NBS at diagnosis ofNHL was nine years. NHL-entities differed from non-AT/NBS-patients: diffuselarge B-cell lymphomas, n = 7 (78%); ALCL, n = 1;lymphoblastic T-cell lymphoma, n = 1. Cervical nodes, paranasalsinuses 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 patientsreceived polychemotherapy according to tumor-entity and stage, none receivedradiation. Dose reductions according to individual tolerance concerned mainlyethotrexate, alkylating agents and epipodophyllotoxines. One patient died oftoxic complications, two patients relapsed and died, one patient suffered fromsecond malignancy. Five of nine patients are in 1. CCR after a medianfolluow-up of five years. Conclusions:Patients with AT and NBS suffer from rare entitiesof pediatric NHL. Curative treatment is possible and should be attempted.Intensity of therapy should be adjusted to individual risk factors andtolerance. Alkylating agents, epipodophyllotoxines should be omitted, dose ofMTX should be limited to 1 g/m2. Further cooperative trials usingstandardized approaches are required.
    Type of Medium: Electronic Resource
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