Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.
The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.
Int J Hematol. 2019 Jun;109(6):737-743. doi: 10.1007/s12185-019-02604-2. Epub 2019 Jan 24.
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 1 week. 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.
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。一名 25 岁男性因伴有多器官功能衰竭的出血倾向、全身性水肿、大量胸腔积液和腹水而被转至我院。他被诊断为 AHA。为了止血，我们为他提供了旁路治疗，并使用环磷酰胺和泼尼松龙来消除可能的抑制剂。然而，他发生了心肺骤停。我们启动了静脉-动脉体外膜肺氧合（VA-ECMO）作为抢救治疗。他的血流动力学状况稳定，并在 1 周内成功脱离 VA-ECMO。我们确认了 FVIII 活性正常且抑制剂消失，旁路治疗停止。然而，全身性水肿伴大量腹水、胸腔积液和肾功能不全持续存在。骨髓活检显示网状纤维蛋白纤维化。这些症状符合 TAFRO 综合征的诊断标准。他接受了托珠单抗（TCZ）治疗，同时逐渐减少了类固醇的剂量。在接受了四个 TCZ 周期的治疗后，TAFRO 综合征的症状逐渐改善。据我们所知，这是首例使用 VA-ECMO 抢救的伴有 AHA 的 TAFRO 综合征的病例。此外，AHA 和 TAFRO 综合征均被 TCZ 很好地控制。
