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Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab.
International Journal of Hematology ( IF 2.1 ) Pub Date : 2019-01-27 , DOI: 10.1007/s12185-019-02604-2
Kei Suzuki 1, 2 , Takeshi Matsumoto 1 , Yoshiaki Iwashita 2 , Ken Ishikura 2 , Masaki Fujioka 2 , Hideo Wada 3 , Naoyuki Katayama 1 , Hiroshi Imai 2
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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.

中文翻译:

用VA-ECMO和tocilizumab成功治疗的并发后天性血友病A和发展为心肺骤停的TAFRO综合征的临床病理特征。

TAFRO综合征和获得性血友病A(AHA)是罕见的威胁生命的疾病;但是,这两种疾病之间的关系尚不清楚。一名25岁的男子因出血倾向并伴有多器官功能衰竭伴全身水肿,大量胸腔积液和腹水而被转入我院。他被诊断出患有AHA。提供了止血旁路疗法和泼尼松龙与环磷酰胺以消除可能的抑制剂。但是,他患有心肺骤停。静脉动脉体外膜氧合(VA-ECMO)开始作为抢救疗法。他的血液动力学状态稳定,并且在1周内从VA-ECMO断奶。我们确认FVIII活性正常且抑制剂消失,并终止了旁路治疗。但是,泛发性水肿伴有大量腹水,胸腔积液和肾功能不全持续存在。骨髓活检显示网蛋白纤维化。这些症状符合TAFRO综合征的诊断标准。他接受了tocilizumab(TCZ),并且类固醇逐渐减少。经过四个周期的TCZ,TAFRO综合征的症状逐渐改善。据我们所知,这是TAFRO综合征伴AHA并由VA-ECMO抢救的首例报告。此外,TCZ可以很好地控制AHA和TAFRO综合征。这是TAFRO综合征伴AHA并由VA-ECMO抢救的首例报道。此外,TCZ可以很好地控制AHA和TAFRO综合征。这是TAFRO综合征伴AHA并由VA-ECMO抢救的首例报道。此外,TCZ可以很好地控制AHA和TAFRO综合征。
更新日期:2019-01-24
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