Kang Mi Yeon, Hahm Jong Ryeal, Jung Tae Sik, Lee Gyeong-Won, Kim Deok Ryong, Park Myoung Hee
Department of Internal Medicine, Gyeongsang National University, Jinju 660-702, Korea.
Yonsei Med J. 2006 Jun 30;47(3):432-6. doi: 10.3349/ymj.2006.47.3.432.
Here we report the case of a 20-year-old female patient previously diagnosed with Hashimoto's thyroiditis and overt hypothyroidism, and who had been taking synthetic thyroxine (100 microg/day) for eight months. She experienced intermittent dizziness and generalized weakness, and was diagnosed as having severe autoimmune hemolytic anemia (AIHA). We prescribed prednisolone treatment and continued synthetic thyroxine administration. Two years and five months later, she developed idiopathic thrombocytopenic purpura (ITP) and was diagnosed with Evans' syndrome. Thereafter, laparoscopic splenectomy was performed because her autoimmune hemolytic anemia was refractory and dependent on steroid therapy. The HLA genotypes of the patient were HLA-A020101/A 2602, HLA-B270502/B5401, HLA-Cw0102/Cw020202, HLA-DRB10404/DRB10405, and HLA-DQB10302/DQ B10401. Hashimoto's thyroiditis is often associated with other nonendocrine autoimmune diseases, and antithyroid antibodies are frequently observed in Evans' syndrome (coexistence of AIHA and ITP). However, there is no report of Evans' syndrome developing in patients with overt hypothyroidism and Hashimoto's thyroiditis. This case suggests that three autoimmune diseases (AIHA, ITP, and Hashimoto's thyroiditis) might share a common immunogenetic pathway in pathogenesis.
在此，我们报告一例20岁女性患者的病例。该患者先前被诊断为桥本甲状腺炎及明显的甲状腺功能减退症，已服用合成甲状腺素（100微克/天）八个月。她出现间歇性头晕和全身乏力，被诊断为患有严重的自身免疫性溶血性贫血（AIHA）。我们给予泼尼松龙治疗，并继续服用合成甲状腺素。两年零五个月后，她患上了特发性血小板减少性紫癜（ITP），并被诊断为伊文氏综合征。此后，由于她的自身免疫性溶血性贫血难治且依赖类固醇治疗，遂进行了腹腔镜脾切除术。该患者的HLA基因型为HLA-A020101/A2602、HLA-B270502/B5401、HLA-Cw0102/Cw020202、HLA-DRB10404/DRB10405以及HLA-DQB10302/DQB10401。桥本甲状腺炎常与其他非内分泌自身免疫性疾病相关，且在伊文氏综合征（AIHA和ITP并存）中经常观察到抗甲状腺抗体。然而，尚无明显甲状腺功能减退症和桥本甲状腺炎患者发生伊文氏综合征的报告。该病例提示，三种自身免疫性疾病（AIHA、ITP和桥本甲状腺炎）在发病机制中可能共享一条共同的免疫遗传途径。
