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Diagnostic dilemma in Evans syndrome

机译:埃文斯综合征的诊断困境

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Evans syndrome, a complex uncommon disease, poses a great diagnostic dilemma due to its variable presentation. The present case of 26-year-old Indian female faced similar difficulty as it was not diagnosed initially when it presented with thrombocytopenia and was treated by platelet concentrate transfusion and steroids. However, the patient showed repeated episodes of remissions and exacerbations without any substantial improvement and later at a tertiary care center it was diagnosed as Evans' Syndrome after direct antiglobulin test and relevant investigations to rule out other causes. This case stresses on the importance of direct antiglobulin test in every patient presenting with autoimmune thrombocytopenia to rule out autoimmune hemolytic anemia and thus excluding this syndrome.
机译:埃文斯综合症(Evans syndrome)是一种罕见的复杂疾病,由于其表现形式多样,因此在诊断上造成了极大的困境。本例26岁的印度女性面临类似的困难,因为最初出现血小板减少症时并没有被诊断出,并接受了血小板浓缩液输注和类固醇治疗。然而,该患者表现出反复发作的发作和加重发作,但没有任何实质性改善,后来在三级护理中心,通过直接抗球蛋白测试和排除其他原因的相关调查,被诊断为埃文斯综合症。该病例强调了直接免疫球蛋白检测在每位自身免疫性血小板减少症患者中排除自身免疫性溶血性贫血并因此排除该综合征的重要性。

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