Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab.

The Korean Journal of Hematology Pub Date : 2011-12-01 Epub Date: 2011-12-27 DOI:10.5045/kjh.2011.46.4.279
Hye Jin Oh, Myung Jae Yun, Seong Tae Lee, Seung June Lee, So Yeon Oh, In Sohn
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引用次数: 13

Abstract

We report a case of a 51-year-old woman with Evans syndrome (autoimmune hemolytic anemia and primary immune thrombocytopenia) and hypothyroidism. She was previously diagnosed with Hashimoto's thyroiditis in 1994 (age, 35) and autoimmune hemolytic anemia (AIHA) 3 years ago. She was treated with oral prednisolone. After a period, in which the anemia waxed and waned, there was an abrupt development of thrombocytopenia (nadir 15×10(9)/L) that coincided with the tapering off of prednisolone after 3 years of administration. Because her thrombocytopenia was refractory to prednisolone, we administered rituximab (375 mg/m(2) weekly) for 4 weeks. Two weeks after the completion of the rituximab treatment, her platelet count was up to 92×10(9)/L. No intermittent peaking of thyroid stimulating hormone occurred after rituximab treatment was initiated. Evans syndrome and autoimmune thyroiditis might share common pathophysiological mechanisms. This notion supports the use of rituximab in a patient suffering from these disorders.

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长期桥本甲状腺炎和利妥昔单抗成功治疗后的Evans综合征。
我们报告一例51岁的妇女埃文斯综合征(自身免疫性溶血性贫血和原发性免疫性血小板减少症)和甲状腺功能减退。她于1994年被诊断为桥本甲状腺炎(35岁),3年前被诊断为自身免疫性溶血性贫血(AIHA)。她接受口服强的松龙治疗。一段时间后,贫血时好时坏,突然出现血小板减少症(最低点15×10(9)/L),同时强的松龙治疗3年后逐渐减少。由于她的血小板减少症对强的松龙是难治性的,我们给她使用了利妥昔单抗(每周375 mg/m(2)),持续4周。利妥昔单抗治疗结束2周后,患者血小板计数达到92×10(9)/L。利妥昔单抗治疗开始后,促甲状腺激素未出现间歇性峰值。Evans综合征和自身免疫性甲状腺炎可能具有共同的病理生理机制。这一观点支持在患有这些疾病的患者中使用利妥昔单抗。
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