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血小板生成素模拟物治疗系统性红斑狼疮或抗磷脂综合征患者难治性免疫性血小板减少性紫癜的安全性和有效性:病例系列

Safety and efficacy of thrombopoeitin mimetics for refractory immune thrombocytopenia purpura in patients with systemic lupus erythematosus or antiphospholipid syndrome: a case series.

作者信息

Lusa A, Carlson A

机构信息

Fontaine Research Park, University of Virginia, USA.

出版信息

Lupus. 2018 Sep;27(10):1723-1728. doi: 10.1177/0961203318770023. Epub 2018 Apr 19.

DOI:10.1177/0961203318770023
PMID:29673280
Abstract

Background While thrombopoeitin (TPO) agonists that act to simulate platelet production have been approved for use in steroid-refractory chronic immune thrombocytopenia purpura (ITP), there are few data on the safety and efficacy of these medications in patients with concurrent systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). Given that these agents can increase all hematopoietic cell lineages, it is unclear if there is an increased risk for exacerbation of the underlying lymphocyte-driven autoimmune disease in this population. Case summaries This case series includes four patients with SLE, one with concurrent APS, who were treated for steroid-refractory ITP with TPO mimetics at the University of Virginia between 2005 and 2015. In three of the four cases the medication was successful in improving platelet counts and preventing bleeding events. In addition, none of the patients experienced thrombosis or worsening of their underlying autoimmune disease. Conclusions This case series suggests that TPO mimetics are safe and moderately effective in patients with ITP in the setting of SLE or APS and do not contribute to increased disease activity.

摘要

背景

虽然用于模拟血小板生成的血小板生成素(TPO)激动剂已被批准用于治疗类固醇难治性慢性免疫性血小板减少性紫癜(ITP),但关于这些药物在合并系统性红斑狼疮(SLE)或抗磷脂综合征(APS)患者中的安全性和有效性的数据却很少。鉴于这些药物可增加所有造血细胞谱系,目前尚不清楚该人群中潜在的淋巴细胞驱动的自身免疫性疾病加重风险是否增加。

病例总结

该病例系列包括4例SLE患者,其中1例合并APS,他们于2005年至2015年在弗吉尼亚大学接受TPO模拟物治疗类固醇难治性ITP。在4例中的3例中,药物成功提高了血小板计数并预防了出血事件。此外,没有患者发生血栓形成或潜在自身免疫性疾病恶化。

结论

该病例系列表明,TPO模拟物在SLE或APS背景下的ITP患者中是安全且中等有效的,并且不会导致疾病活动增加。

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