Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab

  • Kei SuzukiEmail author
  • Takeshi Matsumoto
  • Yoshiaki Iwashita
  • Ken Ishikura
  • Masaki Fujioka
  • Hideo Wada
  • Naoyuki Katayama
  • Hiroshi Imai
Case Report


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 syndrome Acquired hemophilia A Tocilizumab Extracorporeal life support 



The authors thank all colleagues in the Emergency and Critical Center, Mie University Hospital (Drs. K. Ikejiri, R. Esumi, A. Ito, Y. Akama, G. Miyamura, Y. Ieki, E. Kawamoto, K. Yokoyama, Y. Omori, and T. Takeda), and Dr. A. Yamamoto (Department of Emergency Medicine, Mie Prefectural General Medical Center) for their assistance. We thank Ellen Knapp, PhD, from Edanz Group ( for editing a draft of this manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Japanese Society of Hematology 2019

Authors and Affiliations

  1. 1.Department of Hematology and OncologyMie University Graduate School of MedicineTsuJapan
  2. 2.The Emergency and Critical Care CenterMie University HospitalTsuJapan
  3. 3.Department of Molecular and Laboratory MedicineMie University Graduate School of MedicineTsuJapan

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