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Evans综合征与原发性抗磷脂综合征并存的罕见病例

A Rare Case of Simultaneous Evans Syndrome and Primary Antiphospholipid Syndrome.

作者信息

Patel Bhamini P, Jakob John

机构信息

Medicine, Northeast Ohio Medical University, Rootstown, USA.

Hematology/Oncology, Summa Akron City Hospital, Summa Health System, Akron, USA.

出版信息

Cureus. 2020 Feb 1;12(2):e6845. doi: 10.7759/cureus.6845.

Abstract

Evans Syndrome (ES) is a rare autoimmune disorder that presents with simultaneous or sequential development of autoimmune hemolytic anemia (AIHA), thrombocytopenia, and/or neutropenia. This disease may occur in conjunction with other autoimmune disorders. Primary antiphospholipid syndrome (APS) is a disorder characterized by thrombosis, which can cause life-threatening complications such as fetal demise, strokes, or deep vein thrombosis. A 67-year-old male with type 2 diabetes mellitus, hypertension, and renal insufficiency presented with concomitant ES and APS. His hematological abnormalities began in 2013 after a deep vein thrombosis of the left lower extremity led to a diagnosis of APS and was started on chronic warfarin. In 2014, he was found to have immune thrombocytopenia (ITP) with relapses the following year. Several months later, he was hospitalized and diagnosed with AIHA. In the setting of his previous episodes of ITP and current AIHA, the diagnosis of ES was made. The initial treatment was 100 mg prednisone taper, but rituximab was required to make complete platelet recovery. The severe deterioration and rapid recovery with proper treatment of the patient highlights the importance of a timely diagnosis of ES. Mild thrombocytopenia can be associated with APS; however; severe thrombocytopenia may warrant further investigation for other possible causes. Maintaining ES on the differential diagnosis of patients with APS and thrombocytopenia could enhance health outcomes.

摘要

伊文斯综合征(ES)是一种罕见的自身免疫性疾病,其特征为自身免疫性溶血性贫血(AIHA)、血小板减少和/或中性粒细胞减少同时或相继出现。这种疾病可能与其他自身免疫性疾病同时发生。原发性抗磷脂综合征(APS)是一种以血栓形成为特征的疾病,可导致危及生命的并发症,如胎儿死亡、中风或深静脉血栓形成。一名患有2型糖尿病、高血压和肾功能不全的67岁男性同时患有ES和APS。他的血液学异常始于2013年,左下肢深静脉血栓形成后被诊断为APS,并开始使用慢性华法林治疗。2014年,他被发现患有免疫性血小板减少症(ITP),次年复发。几个月后,他住院并被诊断为AIHA。鉴于他之前的ITP发作和目前的AIHA,ES的诊断得以确立。初始治疗为100毫克泼尼松逐渐减量,但需要使用利妥昔单抗才能使血小板完全恢复。患者病情的严重恶化和经适当治疗后的快速恢复凸显了及时诊断ES的重要性。轻度血小板减少可能与APS有关;然而,严重血小板减少可能需要进一步调查其他可能的原因。在对APS和血小板减少患者进行鉴别诊断时考虑到ES,可能会改善健康结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28e7/7053248/12b57cf35f09/cureus-0012-00000006845-i01.jpg

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