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去铁酮相关的骨髓毒性。

Deferiprone-associated myelotoxicity.

作者信息

al-Refaie F N, Wonke B, Hoffbrand A V

机构信息

Department of Haematology, Royal Free Hospital, School of Medicine, London, UK.

出版信息

Eur J Haematol. 1994 Nov;53(5):298-301. doi: 10.1111/j.1600-0609.1994.tb01323.x.

DOI:10.1111/j.1600-0609.1994.tb01323.x
PMID:7813710
Abstract

Agranulocytosis developed in a 63-year-old patient with myelodysplasia 6 weeks after commencing treatment with the oral iron chelator deferiprone (L1, 1,2-dimethyl-3-hydroxypyrid-4-one, CP20) at a daily dose of 79 mg/kg. This was the 3rd case of agranulocytosis (neutrophils 0 x 10(9)/l) in clinical trials of L1 at the Royal Free Hospital. The neutrophil count recovered 7 days after stopping L1 and commencing G-CSF at a dose of 300 micrograms daily. Three other patients with milder degrees of neutropenia (neutrophils < 1.5 x 10(9)/l) have also been observed in our trials. The case histories of these 4 patients are described here; other reported cases of neutropenia or agranulocytosis are reviewed. Based on worldwide long-term clinical trials the incidence of agranulocytosis is about 1.6% and of neutropenia 2%.

摘要

一名63岁的骨髓发育异常患者,在开始口服铁螯合剂去铁酮(L1,1,2 - 二甲基 - 3 - 羟基吡啶 - 4 - 酮,CP20)治疗6周后出现粒细胞缺乏症,每日剂量为79 mg/kg。这是皇家自由医院L1临床试验中第3例粒细胞缺乏症(中性粒细胞0×10⁹/L)病例。停用L1并开始每日剂量为300微克的粒细胞集落刺激因子(G-CSF)治疗7天后,中性粒细胞计数恢复。在我们的试验中还观察到另外3例中性粒细胞减少程度较轻(中性粒细胞<1.5×10⁹/L)的患者。这里描述了这4例患者的病史;并对其他报道的中性粒细胞减少或粒细胞缺乏症病例进行了综述。基于全球长期临床试验,粒细胞缺乏症的发生率约为1.6%,中性粒细胞减少症的发生率为2%。

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Deferiprone-associated myelotoxicity.去铁酮相关的骨髓毒性。
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2
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Deferiprone-induced agranulocytosis: 20 years of clinical observations.去铁酮诱导的粒细胞缺乏症:20年临床观察
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10
Development of oral iron chelator L1.
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Oral nucleic acid therapy using multicompartmental delivery systems.口服核酸治疗的多腔室递药系统。
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Deferiprone-Induced Agranulocytosis : A Critical Review of Five Rechallenged Cases.
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Clin Drug Investig. 1997 Jun;13(6):345-9. doi: 10.2165/00044011-199713060-00007.
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Deferiprone-induced agranulocytosis: 20 years of clinical observations.去铁酮诱导的粒细胞缺乏症:20年临床观察
Am J Hematol. 2016 Oct;91(10):1026-31. doi: 10.1002/ajh.24479. Epub 2016 Aug 4.
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Guidelines for the Standard Monitoring of Patients With Thalassemia: Report of the Thalassemia Longitudinal Cohort.地中海贫血患者标准监测指南:地中海贫血纵向队列报告
J Pediatr Hematol Oncol. 2015 Apr;37(3):e162-9. doi: 10.1097/MPH.0000000000000307.
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