Maeda Sayaka, Matsubara Hiroshi, Hiejima Eitaro, Tanaka Atsushi, Okada Masayuki, Kato Itaru, Umeda Katsutsugu, Hiramatsu Hidefumi, Watanabe Ken-ichiro, Heike Toshio, Adachi Souichi
Department of Pediatrics, Kyoto University Hospital, Kyoto, Japan.
Pediatr Int. 2014 Aug;56(4):626-9. doi: 10.1111/ped.12313.
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.
威尔逊病（WD）是一种细胞铜转运的常染色体隐性缺陷疾病。尽管急性淋巴细胞白血病（ALL）是儿童恶性肿瘤最常见的形式，但迄今为止，仅有两例ALL与WD相关的病例报道。一名患者死于复发和感染，另一名患者在治疗期间死于中性粒细胞减少性败血症。我们在此描述一名患有WD和ALL的10岁女孩的病例。化疗的不良事件，包括肝毒性和严重的骨髓抑制，需要调整化疗剂量。治疗完成后，该患者ALL持续缓解，且肝脏损伤未进展，已达2年。对于WD患者进行化疗时，应考虑严重的治疗相关毒性。
