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A strange case of Evans syndrome

机译：埃文斯综合征的奇怪情况

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Evans syndrome is a rare autoimmune disease presenting hemolytic anemia, thrombocytopenia and/or neutropenia. It may be associated with other autoimmune or lymphoproliferative diseases. It can have an extremely serious disease course and, in rare cases, this can even be life-threatening. First-line treatment consists of steroids and/or immunoglobulin. Further therapy with rituximab, vincristine, cyclophosphamide and other immunosuppressive drugs can be considered in unresponsive patients. We report a case of Evans syndrome in a 54-year old woman admitted to the Emergency Department (ED) for asthenia. Etiopathogenic, clinical, therapeutic and evolutive aspects are discussed. In contrast to many cases described in the literature, our patient had a satisfactory response to corticoids. We also discuss how to make a specific diagnosis, even in a suburban ED with limited resources, in order to admit patients to the appropriate hospital department and allow the correct therapy to be started as early as possible.
机译：evans综合征是一种罕见的自身免疫性疾病，呈现出溶血性贫血，血小板减少症和/或中性粒细胞病。它可能与其他自身免疫或淋巴抑制性疾病有关。它可以具有极其严重的疾病课程，并且在极少数情况下，这甚至可以是危及生命的。一线治疗包括类固醇和/或免疫球蛋白。在无响应的患者中可以考虑进一步治疗Rituximab，血管内，环磷酰胺和其他免疫抑制药物。我们举报了一个54岁的女性中埃文斯综合征的案例，该妇女承认急诊部门（ED）的哮喘。讨论了病因致病，临床，治疗和演化方面。与文献中描述的许多病例相比，我们的患者对皮质糖有令人满意的反应。我们还讨论了如何进行特定诊断，即使在具有有限的资源的郊区，为了承认患者到适当的医院部门，并尽早开始进行正确的治疗。

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