title: Pilot study of modified LMB-based therapy for children with ataxia-telangiectasia and advanced stage high grade mature B-cell malignancies.
abstract: Children with ataxia-telangiectasia (A-T) and cancer have a poorer prognosis due in part to increased treatment-related toxicity. We piloted a curative intent approach in five children with A-T who presented with advanced stage (III, n = 2; IV, n = 3) B-NHL (diffuse large B-cell lymphoma, n = 4; Burkitt leukemia, n = 1) using a modified LMB-based protocol. Two achieved sustained CCR (one, CCR at 6 years; one, pulmonary death after 3 years in CCR). Two died from toxicity during induction and 1 failed induction with progressive disease. Novel therapeutic approaches which overcome drug resistance and are less toxic are needed for children with A-T and B-NHL.
authors.affiliation: Departments of Oncology, St. Jude Children's Research Hospital Memphis, Tennessee.
authors.forename: J T
authors.initials: JT
authors.lastname: Sandlund
authors.forename: M M
authors.initials: MM
authors.lastname: Hudson
authors.forename: W
authors.initials: W
authors.lastname: Kennedy
authors.forename: M
authors.initials: M
authors.lastname: Onciu
authors.forename: M B
authors.initials: MB
authors.lastname: Kastan
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001260:Ataxia Telangiectasia / Q000150:complications / Q000188:drug therapy*; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D003561:Cytarabine / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D002955:Leucovorin / Q000627:therapeutic use; D016393:Lymphoma, B-Cell / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D009367:Neoplasm Staging; D010865:Pilot Projects; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D011446:Prospective Studies; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D003561:Cytarabine; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D002955:Leucovorin; D011241:Prednisone; D006854:Hydrocortisone; D008727:Methotrexate
keywords: Hodgkin lymphoma; ataxia-telangiectasia; children; non-Hodgkin lymphoma
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