Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab
Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab
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DOI：
10.1007/s12185-019-02604-2
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发表时间：
2019-06-01
影响因子：
2.1
通讯作者：
Imai, Hiroshi
中科院分区：
文献类型：
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作者：
Suzuki, Kei;Matsumoto, Takeshi;Imai, Hiroshi
TAFRO综合征和获得性血友病A（AHA）是罕见的，危及生命的疾病;然而，这两种疾病之间的关系尚不清楚。一位25岁男性因出血倾向合并多器官功能衰竭伴全身水肿、大量胸腔积液和腹水而转入我院。他被诊断为AHA。提供旁路止血治疗和环磷酰胺联合泼尼松龙根除可能的抑制剂。然而，他遭受了心肺骤停。开始静脉动脉体外膜肺氧合（VA-ECMO）作为补救治疗。患者血流动力学状态稳定，1周后脱离VA-ECMO。我们证实正常的FVIII活性和抑制剂的消失，并停止旁路治疗。然而，全身水肿伴大量腹水、胸腔积液和肾功能不全持续存在。骨髓活检显示网硬蛋白纤维化。这些症状符合TAFRO综合征的诊断标准。患者接受托珠单抗（TCZ）治疗，类固醇逐渐减少。TCZ治疗4个周期后，TAFRO综合征症状逐渐改善。据我们所知，这是TAFRO综合征伴AHA并经VA-ECMO抢救的首例报告。此外，TCZ对AHA和TAFRO综合征的控制良好。
TAFRO syndrome and acquired hemophilia A (AHA) are rare, life-threatening diseases; however, the relationship between these two diseases is unknown. A 25-year-old man was transferred to our hospital because of bleeding tendency accompanied by multiple organ failure with generalized edema, massive pleural effusion, and ascites. He was diagnosed with AHA. Bypass therapy for hemostasis and cyclophosphamide with prednisolone to eradicate possible inhibitors were provided. However, he suffered from cardiopulmonary arrest. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated as rescue therapy. His hemodynamic status stabilized and he was weaned from VA-ECMO in 1week. We confirmed normal FVIII activity and disappearance of the inhibitor, and bypass therapy was discontinued. However, generalized edema with massive ascites, pleural effusion, and renal insufficiency persisted. Bone marrow biopsy showed reticulin fibrosis. These symptoms fulfilled the diagnostic criteria of TAFRO syndrome. He received tocilizumab (TCZ) and steroid was tapered off. After four cycles of TCZ, symptoms of TAFRO syndrome gradually improved. To the best of our knowledge, this is the first report of TAFRO syndrome accompanied by AHA with rescue by VA-ECMO. Additionally, AHA and TAFRO syndrome were well controlled by TCZ.