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艾曲泊帕作为系统性红斑狼疮中免疫性血小板减少性紫癜的激素节约疗法。

Eltrombopag as steroid sparing therapy for immune thrombocytopenic purpura in systemic lupus erythematosus.

作者信息

Maroun M-C, Ososki R, Andersen J C, Dhar J P

机构信息

Internal Medicine Department, Rheumatology Division, Wayne State University, Detroit, MI, USA

Medicine and Oncology Department, Wayne State University School of Medicine, Karmanos Cancer Institute, USA.

出版信息

Lupus. 2015 Jun;24(7):746-50. doi: 10.1177/0961203314559632. Epub 2014 Nov 21.

Abstract

BACKGROUND

Eltrombopag activates the thrombopoietin (TPO) surface receptor on the megakaryocyte, which increases the production of platelets, and rapidly improves circulating platelet numbers in patients with immune thrombocytopenic purpura (ITP). This allows for rapid tapering and/or cessation of corticosteroid therapy. Less is known about the platelet response to this drug in ITP associated with systemic lupus erythematosus (SLE).

METHODS

A retrospective review was performed of the clinical course of three consecutive patients, each with SLE-associated ITP who were initially treated with corticosteroids or other immunomodulatory therapy. These patients were treated with eltrombopag at the DMC Center for Bleeding Disorders and Thrombosis. Eltrombopag was administered according the package insert, with an initial dose of 50 mg daily, with weekly, then monthly monitoring of platelet counts and dose adjustments. Some immunomodulatory agents (e.g. hydroxychloroquine) were continued to control non hematologic SLE manifestations.

RESULTS

All three patients maintained acceptable platelet counts (>50,000/mm(3) for >3 years) following tapering and cessation of corticosteroids. The drug was well-tolerated and there were no adverse events, and specifically no thrombotic events.

CONCLUSION

Eltrombopag is effective as a rapidly acting corticosteroid sparing therapy for patients with ITP associated with SLE. This is important in reducing corticosteroid related side effects and morbidities in treating SLE patients with ITP. Larger studies are needed to ascertain safety and efficacy of eltrombopag in SLE patients with ITP, particularly those with coexisting antiphospholipid antibodies.

摘要

背景

艾曲波帕可激活巨核细胞上的血小板生成素(TPO)表面受体,从而增加血小板的生成,并迅速提高免疫性血小板减少性紫癜(ITP)患者的循环血小板数量。这使得能够迅速减少和/或停用皮质类固醇疗法。对于与系统性红斑狼疮(SLE)相关的ITP患者对这种药物的血小板反应了解较少。

方法

对连续3例最初接受皮质类固醇或其他免疫调节治疗的SLE相关ITP患者的临床病程进行回顾性分析。这些患者在DMC出血性疾病和血栓形成中心接受艾曲波帕治疗。艾曲波帕按照药品说明书给药,初始剂量为每日50毫克,每周监测血小板计数,然后每月监测并调整剂量。继续使用一些免疫调节药物(如羟氯喹)来控制非血液学的SLE表现。

结果

所有3例患者在减少和停用皮质类固醇后均维持了可接受的血小板计数(>50,000/mm³超过3年)。该药物耐受性良好,未发生不良事件,尤其是未发生血栓形成事件。

结论

艾曲波帕作为一种快速起效的皮质类固醇节省疗法,对SLE相关ITP患者有效。这对于减少治疗SLE合并ITP患者时皮质类固醇相关的副作用和发病率具有重要意义。需要进行更大规模的研究来确定艾曲波帕在SLE合并ITP患者中的安全性和有效性,尤其是那些同时存在抗磷脂抗体的患者。

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