title: Malignant non-Hodgkin's lymphoma of childhood and adolescence in Austria--therapy results between 1986 and 2000.
abstract: Between 1986 and 2000 183 Austrian children and adolescents with non-Hodgkin's lymphoma (NHL) and mature B-cell acute leukemia (B-ALL) were enrolled in 3 consecutive studies of the Berlin-Frankfurt-Münster (BFM) Group. In trial NHL-BFM 86, patients were stratified according to the histologic subtype and clinical stage. In the succeeding studies NHL-BFM 90 and 95, treatment stratification was additionally based on the speed of tumor response to therapy and for children with B-cell NHL/B-ALL also on the pre-therapeutic serum lactic dehydrogenase level. Event-free survival rates were 84% +/- 6% in trial NHL-BFM 86 (n = 39) and 86% +/- 4% in both trials NHL-BFM 90 (n = 67) and NHL-BFM 95 (n = 77). Patients with lymphoblastic lymphoma (mainly with T-cell phenotypes) had an excellent prognosis with an ALL-type chemotherapy regimen (n = 49; relapse, n = 1), whereas an intensive, short-pulse therapy delivered within a 2- to 4-month period was found to be highly efficacious in children with B-cell NHL/B-ALL (n = 114; relapse, n = 6; progression, n = 5). Patients with anaplastic large cell lymphoma (ALCL) who were treated with similar alternating short courses of multi-agent chemotherapy had a less good outcome (n = 20; relapse, n = 6, progression, n = 3). Children with B-cell NHL and B-ALL who failed initial therapy also had a dismal prognosis (10/11 patients died). Local radiotherapy as a part of lymphoma therapy was completely abandoned in study NHL-BFM 90 and surgical interventions were confined to specific situations such as complete resection in localized B-cell NHL and ALCL, diagnostic biopsy and second-look operation. In conclusion, our results showed that the BFM treatment strategy for lymphoblastic lymphoma and B-cell NHL/B-ALL was highly successful in the majority of patients; however, optimal treatment for children with ALCL has not yet been defined. As a consequence, larger trials at an international level are necessary to find new prognostic markers that might define more precisely those patients who need further intensification of first-line treatment or novel therapy.
authors.affiliation: St. Anna Children's Hospital, Vienna, Austria.
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Georg
authors.initials: G
authors.lastname: Mann
authors.forename: Michael
authors.initials: M
authors.lastname: Dworzak
authors.forename: Monika
authors.initials: M
authors.lastname: Trebo
authors.forename: Christian
authors.initials: C
authors.lastname: Urban
authors.forename: Franz-Martin
authors.initials: FM
authors.lastname: Fink
authors.forename: Ernst
authors.initials: E
authors.lastname: Horcher
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Hansjörg
authors.initials: H
authors.lastname: Riehm
authors.forename: Helmut
authors.initials: H
authors.lastname: Gadner
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002051:Burkitt Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003627:Data Interpretation, Statistical; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000628:therapy; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000628:therapy*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents