Relapsing Evans syndrome (ES) and systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS) is very rare association. Coexistence of these syndromes is potentially fatal and require high-dose combined immunosuppressive therapy. We describe a case of successful use of Bortezomib and plasma exchange in a patient with ES and APS refractory to standard therapy. Thirty-two-year-old male who presented episodes of relapsing hemolytic anemia, pancytopenia and multiple thrombosis with positive direct and indirect antiglobulin test result, lupus anticoagulant and medium titer of anti-beta-2-glycoprotein 1 and anti-cardiolipin antibodies was diagnosed with ES and SLE with secondary APS. High-dose therapy by steroids and Cyclosporin A were started with temporary improvement. There was also no stable improvement with Rituximab and Cyclophosphamide. Bortezomib in combination with cyclosporine A and plasma exchange was introduced. He had stable improvement in hematological parameters with no evidence of relapse of hemolytic crisis or thrombosis during a follow-up for 1 year.

复发性伊文斯综合征（ES）和系统性红斑狼疮（SLE）与继发性抗磷脂综合征（APS）的关联非常罕见。这些综合征的共存可能致命，需要大剂量联合免疫抑制治疗。我们描述了在标准治疗难治的ES和APS患者中成功使用硼替佐米和血浆置换的案例。一名32岁男性患者，该患者表现为复发性溶血性贫血，全血细胞减少症和多发性血栓形成，直接和间接抗球蛋白测试结果均为阳性，诊断出狼疮抗凝药和抗β-2-糖蛋白1和抗心磷脂抗体的中等滴度ES和SLE以及辅助APS。类固醇和环孢菌素A的大剂量治疗开始出现暂时改善。利妥昔单抗和环磷酰胺也没有稳定的改善。引入硼替佐米联合环孢霉素A和血浆置换。随访1年，他的血液学参数稳定改善，没有溶血危险或血栓形成复发的迹象。