title: Chemo- and radiosensitivity testing in a patient with ataxia telangiectasia and Hodgkin disease.
abstract: Treatment of Hodgkin disease (HD) in ataxia telangiectasia (AT) patients is hampered by hypersensitivity to radiation and chemotherapy. Most patients die, due to toxicity or, rarely, to progressive disease. The authors report on a 9-year-old girl with stage IIA HD and AT She was treated with a tailored combined modality approach. No unacceptable toxicity was found, but the girl died of a relapse outside the irradiation field. In comparison with fibroblasts of non-AT patients, the fibroblasts of the patient were 3 times as sensitive for radiotherapy but just 1.2 times as sensitive for doxorubicin. A good correlation was shown between in vitro radio- and chemosensitivity testing and the observed clinical toxicity. The authors suggest, therefore, treating AT patients as much as possible according to standard protocols by adjusting the radiotherapy delivery and the chemotherapy regimen to individual doses derived from in vitro radio- and chemosensitivity testing.
authors.affiliation: Department of Pediatrics, University Hospital Groningen, Beatrix Children's Hospital, The Netherlands. r.y.j.tamminga@bkk.azg.nl
authors.forename: R Y J
authors.initials: RY
authors.lastname: Tamminga
authors.forename: W V
authors.initials: WV
authors.lastname: Dolsma
authors.forename: J A
authors.initials: JA
authors.lastname: Leeuw
authors.forename: H H
authors.initials: HH
authors.lastname: Kampinga
mesh_terms: D000970:Antineoplastic Agents / Q000633:toxicity*; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D002470:Cell Survival / Q000187:drug effects / Q000528:radiation effects; D002648:Child; D003131:Combined Modality Therapy / Q000009:adverse effects; D004307:Dose-Response Relationship, Radiation; D017809:Fatal Outcome; D005260:Female; D005347:Fibroblasts / Q000166:cytology / Q000187:drug effects / Q000528:radiation effects; D006689:Hodgkin Disease / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D006801:Humans; D007963:Leukocytes, Mononuclear / Q000166:cytology / Q000187:drug effects / Q000528:radiation effects; D011878:Radiotherapy / Q000009:adverse effects*
chemical_list: D000970:Antineoplastic Agents