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难治性免疫性血小板减少症伴抗磷脂抗体患者的多灶性骨无菌性坏死;病例报告及文献复习。

Multifocal avascular necrosis in a patient with refractory immune thrombocytopenia and antiphospholipid antibodies; case report and review of literature.

机构信息

a Rheumatology & Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine , Cairo University , Cairo , Egypt.

b Division of Rheumatology, Department of Medicine , University of Alabama at Birmingham , Birmingham , AL , USA.

出版信息

Platelets. 2019;30(5):664-671. doi: 10.1080/09537104.2018.1535701. Epub 2018 Oct 29.

Abstract

Avascular necrosis (AVN) is a devastating condition that is rarely reported in patients with immune thrombocytopenia (ITP). Treatment with steroids remains a major risk factor for developing AVN. However, the incidence of AVN in patients with ITP requiring corticosteroid therapy is much less than that observed with other clinical conditions requiring corticosteroids. ITP is a bleeding disorder but can be also be a pro-thrombotic state via different mechanisms and thus could result in AVN. Among the possible causes of this pro-thrombotic state is the presence of antiphospholipid antibodies (aPLs). In this case, we report a patient with refractory ITP who developed multifocal AVN around the time she acquired new aPLs. We also discuss different mechanisms by which risk of thrombosis is increased in ITP and the relationship between ITP, aPLs and antiphospholipid syndrome.

摘要

骨坏死(AVN)是一种破坏性疾病,在免疫性血小板减少症(ITP)患者中很少见。类固醇治疗仍然是发生 AVN 的主要危险因素。然而,需要皮质类固醇治疗的 ITP 患者发生 AVN 的发生率远低于其他需要皮质类固醇治疗的临床情况。ITP 是一种出血性疾病,但也可能通过不同的机制成为血栓形成状态,从而导致 AVN。导致这种血栓形成状态的可能原因之一是存在抗磷脂抗体(aPL)。在这种情况下,我们报告了一例难治性 ITP 患者,她在获得新的 aPL 时发生了多处 AVN。我们还讨论了 ITP 中血栓形成风险增加的不同机制以及 ITP、aPL 和抗磷脂综合征之间的关系。

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