自身免疫性甲状腺炎患者的埃文斯综合征--一种罕见的联系

Jeyapraniya Arumugam, Shamila De Silva
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摘要

一名 46 岁的女性被诊断患有甲状腺功能减退症,每天服用 100 微克的合成甲状腺素治疗一年。她主诉最近出现劳力性呼吸困难,并伴有乏力、嗜睡和胃口不好。检查发现她有严重贫血和血小板严重减少,需要输血。她患有库姆斯阳性自身免疫性溶血性贫血和免疫性血小板减少性紫癜,确诊为埃文斯综合征。她的抗核抗体检测也呈阳性,但没有发现其他明显的自身免疫性疾病,这表明她被诊断为原发性埃文斯综合征。患者接受了为期四天的静脉注射地塞米松治疗,随后口服泼尼松龙(1 毫克/千克/天),并补充钙和维生素 D。甲状腺过氧化物酶抗体呈阳性,甲状腺闪烁扫描摄取减少,确诊为自身免疫性甲状腺炎。桥本氏甲状腺炎经常与非内分泌自身免疫性疾病并存。埃文斯综合征是一种同时出现自身免疫性溶血性贫血、细胞减少症和免疫性血小板减少性紫癜的病症,有时可观察到抗甲状腺抗体的存在。然而,在明显甲状腺功能减退症和桥本氏甲状腺炎患者中出现埃文斯综合征的病例并不多见。这个病例意味着,自身免疫性溶血性贫血、免疫性血小板减少性紫癜和桥本氏甲状腺炎这三种不同的自身免疫性疾病在发病机制上可能存在着共同的免疫遗传途径。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Evans Syndrome in a Patient with Auto Immune Thyroiditis – A Rare Association
A 46-year-old woman, diagnosed with hypothyroidism, was on treatment with synthetic thyroxine 100 micrograms per day for one year. She complained of recent-onset exertional dyspnoea, and had malaise, lethargy, and pica. Investigations revealed significant anaemia and severe thrombocytopenia, necessitating blood transfusion. She had Coombs-positive autoimmune haemolytic anaemia and immune thrombocytopenic purpura, confirming a diagnosis of Evans syndrome. She also had a positive antinuclear antibody test but had no other notable findings of autoimmune disease, suggesting a diagnosis of primary Evans syndrome. The patient was treated with intravenous dexamethasone for four days, followed by oral prednisolone at 1mg/kg/ day, with supplemental calcium and vitamin D. Thyroperoxidase antibody was positive and there was reduced uptake in thyroid scintigraphy, confirming a diagnosis of autoimmune thyroiditis. Hashimoto’s thyroiditis frequently coexists with non-endocrine autoimmune disorders. Presence of antithyroid antibodies is sometimes observed in Evans syndrome, a condition where there is simultaneous occurrence of autoimmune Haemolytic Anaemia, Cytopoenia and immune thrombocytopenic purpura. However, the development of Evans syndrome in patients with overt hypothyroidism and Hashimoto’s thyroiditis is rare. This case implies the potential existence of a shared immunogenetic pathway among three distinct autoimmune diseases, namely autoimmune haemolytic anemia, immune thrombocytopenic purpura, and Hashimoto’s thyroiditis, in the pathogenesis.
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