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. Int J Hematol. 2019 Jan 24;: Authors: Suzuki K, Matsumoto T, Iwashita Y, Ishikura K, Fujioka M, Wada H, Katayama N, Imai H Abstract 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...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research