破解埃文斯之谜:与 Sjogren 和系统性红斑狼疮的罕见关联

Gyan Prakash Kumar, H. M. F. Bari, Piyush Ratan, Tanya Mishra, Ashutosh Kumar, P. N. Jha, Ashok Kumar
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引用次数: 0

摘要

埃文斯综合征(ES)的特点是同时或连续出现温热性自身免疫性溶血性贫血(AIHA)和免疫性血小板减少症(ITP),以及较少见的自身免疫性中性粒细胞减少症。它可能自发出现,也可能是自身免疫性疾病、恶性肿瘤或淋巴增生性疾病的结果。临床表现可能与溶血和血小板减少有关,可能导致危及生命的后果。ES 是一种罕见的排除性诊断。由于其发病率较低,治疗通常是经验性的,主要依靠静脉注射皮质类固醇激素或免疫球蛋白。我们现在介绍的病例是一名 46 岁的女性,口腔和牙龈出血,全身出现皮疹,之前未确诊为风湿病。该病例具有关键意义,因为它突出了导致 ES 的一个关键因素,并提出了解决该病症的实用方法。
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Cracking of enigma of Evans: a rare association with Sjogren and systemic lupus erythematosus
Evans syndrome (ES) is characterized by the simultaneous or consecutive occurrence of warm autoimmune hemolytic anemia (AIHA) along with immune thrombocytopenia (ITP), and less commonly, autoimmune neutropenia. It may manifest spontaneously or as a result of autoimmune, malignancy or lymphoproliferative disease. Clinical manifestations may be associated with hemolysis and thrombocytopenia, potentially leading to life-threatening outcomes. ES is a rare diagnosis of exclusion. Due to its infrequency, the treatment is typically empirical, relying largely on intravenous corticosteroids or immunoglobulins. We are presenting case of a 46-year-old- female with bleeding from the mouth and gums and rashes all over the body with no prior diagnosis of rheumatological disorder. This case is pivotal as it highlights a key factor contributing to ES and presents a pragmatic method for addressing the condition.
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