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

Background: Lymphoma ana leukemia are the commonest malignant diseases in p atients with chromosomal breakage syndromes and immunodeficiency (Ataxia te leangiectasia (AT) and Nijmegen breakage syndrome (NBS)). With improved man agement of infections, malignant disease is more frequently diagnosed and h as become one of the commonest causes of death in pediatric AT and NBS. Patients and methods: In three consecutive multicenter therapy trials for p ediatric non-Hodgkin's lymphoma (NHL) (NHL-BFM), 1569 patients with newly d iagnosed 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 nin e years. NHL-entities differed from non-AT/NBS-patients: diffuse large B-ce ll lymphomas, n = 7 (78%); ALCL, n = 1; lymphoblastic T-cell lymphoma, n = 1. Cervical nodes, paranasal sinuses and epipharynx were the sites most fre quently involved. Stages were: I and II in three patients, III in five and IV in one patient. All patients received polychemotherapy according to tumo r-entity and stage, none received radiation. Dose reductions according to i ndividual tolerance concerned mainly ethotrexate, alkylating agents and epi podophyllotoxines. One patient died of toxic complications, two patients re lapsed and died, one patient suffered from second malignancy. Five of nine patients are in 1. CCR after a median follow-up of five years. Conclusions: Patients with AT and NBS suffer from rare entities of pediatri c NHL. Curative treatment is possible and should be attempted. Intensity of therapy should be adjusted to individual risk factors and tolerance. Alkyl ating agents, epipodophyllotoxines should be omitted, dose of MTX should be limited to 1 g/m(2). Further cooperative trials using standardized approac hes are required.