Rutherford Megan, Tran Minh, Salazar Leonardo, Iqbal Fatima, Mazharuddin Anam, Camarena Julieanna
Department of Internal Medicine, University of Texas Medical Branch, Galveston, USA.
Department of Pathology and Laboratory Medicine, University of Texas Medical Branch, Galveston, USA.
Cureus. 2021 Jul 27;13(7):e16658. doi: 10.7759/cureus.16658. eCollection 2021 Jul.
Evans syndrome is a rare autoimmune disorder where patients develop autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and less commonly immune neutropenia. Patients typically present with fatigue, pallor, jaundice, petechiae, or epistaxis. A 27-year-old man with a history of atopic dermatitis for which he recently began treatment with dupilumab presented to the emergency department with a headache and blurry vision. Multiple Roth spots were seen on fundoscopic examination. Laboratory studies were consistent with warm AIHA, confirmed by a positive direct antiglobulin test (DAT), and severe thrombocytopenia. He was diagnosed with Evans syndrome. He was treated with corticosteroids, rituximab, and intravenous immunoglobulin (IVIG). His recovery was prolonged with the slow improvement of anemia and thrombocytopenia. This is an atypical presentation of Evans syndrome with isolated symptoms of new-onset blurry vision and headache along with the finding of Roth spots. Another interesting feature in the case is the recent use of dupilumab. Dupilumab is a monoclonal antibody that inhibits the T-helper cells type 2 (Th2) signaling pathway by blocking interleukin (IL)-4 and IL-13 binding. This alteration in the immune response could have a role in the development of Evans syndrome.
伊文斯综合征是一种罕见的自身免疫性疾病，患者会出现自身免疫性溶血性贫血（AIHA）、免疫性血小板减少症（ITP），较少见的还有免疫性中性粒细胞减少症。患者通常表现为疲劳、面色苍白、黄疸、瘀点或鼻出血。一名27岁有特应性皮炎病史的男性，近期开始使用度普利尤单抗治疗，因头痛和视力模糊到急诊科就诊。眼底检查发现多个罗特斑。实验室检查结果与温抗体型自身免疫性溶血性贫血相符，直接抗球蛋白试验（DAT）呈阳性确诊，同时伴有严重血小板减少症。他被诊断为伊文斯综合征。给予他皮质类固醇、利妥昔单抗和静脉注射免疫球蛋白（IVIG）治疗。随着贫血和血小板减少症的缓慢改善，他的康复过程延长。这是伊文斯综合征的非典型表现，伴有新发视力模糊和头痛的孤立症状以及罗特斑的发现。该病例的另一个有趣特征是近期使用了度普利尤单抗。度普利尤单抗是一种单克隆抗体，通过阻断白细胞介素（IL）-4和IL-13的结合来抑制2型辅助性T细胞（Th2）信号通路。这种免疫反应的改变可能在伊文斯综合征的发生中起作用。
