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难治性免疫性血小板减少性紫癜与巨细胞病毒感染:呼吁改变现行指南

Refractory Immune Thrombocytopenic Purpura and Cytomegalovirus Infection: A Call for a Change in the Current Guidelines.

作者信息

Shimanovsky Alexei, Patel Devbala, Wasser Jeffrey

机构信息

Department of Hematology and Oncology, University of Connecticut Health Center, 263 Farmington Ave, Farmington, CT 06030.

Department of Pathology and Laboratory Services, Manchester Memorial Hospital, 71 Haynes Street, Manchester, CT 06040.

出版信息

Mediterr J Hematol Infect Dis. 2016 Jan 1;8(1):e2016010. doi: 10.4084/MJHID.2016.010. eCollection 2016.

Abstract

Immune thrombocytopenic purpura (ITP) is characterized by a decreased platelet count caused by excess destruction of platelets and inadequate platelet production. In many cases, the etiology is not known, but the viral illness is thought to play a role in the development of some cases of ITP. The current (2011) American Society of Hematology ITP guidelines recommend initial diagnostic studies to include testing for HIV and Hepatitis C. The guidelines suggest that initial treatment consist of observation, therapy with corticosteroids, IVIG or anti D. Most cases respond to the standard therapy such that the steroids may be tapered and the platelet counts remain at a hemostatically safe level. Some patients with ITP are dependent on long-term steroid maintenance, and the thrombocytopenia persists with the tapering of the steroids. Recent case reports demonstrate that ITP related to cytomegalovirus (CMV) can persist in spite of standard therapy and that antiviral therapy may be indicated. Herein we report a case of a 26-year-old female with persistent ITP that resolved after the delivery of a CMV-infected infant and placenta. Furthermore, we review the current literature on CMV-associated ITP and propose that the current ITP guidelines be amended to include assessment for CMV, even in the absence of signs and symptoms, as part of the work-up for severe and refractory ITP, especially prior to undergoing an invasive procedure such as splenectomy.

摘要

免疫性血小板减少性紫癜(ITP)的特征是血小板计数减少,这是由血小板过度破坏和血小板生成不足所致。在许多情况下,病因不明,但病毒感染被认为在某些ITP病例的发病过程中起作用。美国血液学会2011年发布的ITP指南建议,初始诊断研究应包括检测HIV和丙型肝炎。该指南指出,初始治疗包括观察、使用皮质类固醇、静脉注射免疫球蛋白(IVIG)或抗D治疗。大多数病例对标准治疗有反应,这样类固醇可以逐渐减量,血小板计数维持在止血安全水平。一些ITP患者依赖长期类固醇维持治疗,随着类固醇减量,血小板减少症仍会持续。最近的病例报告表明,尽管进行了标准治疗,与巨细胞病毒(CMV)相关的ITP仍可能持续存在,可能需要进行抗病毒治疗。在此,我们报告一例26岁女性持续性ITP患者,在分娩出感染CMV的婴儿和胎盘后病情缓解。此外,我们回顾了目前关于CMV相关ITP的文献,并建议修订当前的ITP指南,将CMV评估纳入其中,即使在没有症状和体征的情况下,作为重症和难治性ITP检查的一部分,尤其是在进行脾切除术等侵入性手术之前。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b96d/4696470/79f1bfed46f8/mjhid-8-1-e2016010f1.jpg

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