title: Successful treatment of acute myeloid leukaemia in a patient with ataxia telangiectasia.
abstract: Ataxia telangiectasia (AT) is a rare autosomal recessive multisystem disorder characterised by cerebellar degeneration, immunodeficiency and cancer predisposition. Around 10% of AT patients develop lymphoid malignancies, but the development of myeloid leukaemia with AT (AT-AML) is extremely rare, and there have been no previous publications regarding suitable therapies. Here, we first describe a successful therapeutic experience in a patient with AT-AML (FAB-M1) who attained remission after induction therapy and maintained stable disease for a year. To minimise therapy-induced toxicity, low-dose induction was applied first, though this was obviously insufficient and the patient subsequently responded well to dose-intensified short-term chemotherapy. In this report, we suggest a curative therapeutic approach for AT-AML, though the issue of how best to manage patients with cancer complicated by immunodeficiency remains undecided.
authors: Department of Paediatrics, Yamagata University Faculty of Medicine, Yamagata, Japan.
authors: Tadashi
authors: T
authors: Onoda
authors: Miyako
authors: M
authors: Kanno
authors: Toru
authors: T
authors: Meguro
authors: Hiroko
authors: H
authors: Sato
authors: Noriyuki
authors: N
authors: Takahashi
authors: Takako
authors: T
authors: Kawakami
authors: Tetsuo
authors: T
authors: Mitsui
authors: Kiyoshi
authors: K
authors: Hayasaka
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000175:diagnosis; D020732:Cytogenetic Analysis; D004334:Drug Administration Schedule; D005434:Flow Cytometry; D042822:Genomic Instability; D006801:Humans; D060828:Induction Chemotherapy; D007621:Karyotyping; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D016896:Treatment Outcome
keywords: acute myeloid leukaemia; ataxia telangiectasia; reduced-dose chemotherapy