title: Acute lymphoblastic leukemia in a girl with Wilson's disease.
abstract: Wilson's disease (WD) is an autosomal recessive defect in cellular copper transportation. Although acute lymphoblastic leukemia (ALL) is the most common form of childhood malignancy, only two cases of ALL associated with WD have been reported to date. One patient died of relapse and infection, and the other died of neutropenic sepsis during the treatment. We here describe the case of a 10-year-old girl with WD and ALL. Adverse events of chemotherapy, including liver toxicity and severe myelosuppression, necessitated adjustments in the chemotherapy doses. After completion of the treatment, the patient has remained in remission from ALL without progression of liver damage for 2 years. Severe treatment-related toxicity should be considered in chemotherapy for patients with WD.
authors.affiliation: Department of Pediatrics, Kyoto University Hospital, Kyoto, Japan.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Maeda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Matsubara
authors.forename: Eitaro
authors.initials: E
authors.lastname: Hiejima
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.forename: Masayuki
authors.initials: M
authors.lastname: Okada
authors.forename: Itaru
authors.initials: I
authors.lastname: Kato
authors.forename: Katsutsugu
authors.initials: K
authors.lastname: Umeda
authors.forename: Hidefumi
authors.initials: H
authors.lastname: Hiramatsu
authors.forename: Ken-ichiro
authors.initials: K
authors.lastname: Watanabe
authors.forename: Toshio
authors.initials: T
authors.lastname: Heike
authors.forename: Souichi
authors.initials: S
authors.lastname: Adachi
mesh_terms: D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy
keywords: ATP7B; d-penicillamine; glutathione-S-transferase; liver toxicity; zinc acetate