複数回の経カテーテル的動脈塞栓術を含む集学的治療により救命しえた後天性血友病Aの1例

抄録

Acquired hemophilia A (AHA), caused by expression of factor VIII (FVIII) inhibitors, is a rare disease associated with severe coagulopathy and massive bleeding. A woman in her 70s pre-sented at our emergency department with lateral chest subcutaneous hemorrhage. Blood tests revealed anemia and prolonged activated partial thromboplastin time (APTT), and FVIII ac-tivity was markedly reduced, and so AHA was diagnosed. Blood transfusions did not improve the anemia. Angiography was performed to identify the bleeding source. We identified the point of extravascular leakage of contrast medium at the left intercostal arteries and performed transcatheter arterial embolization (TAE). On hospitalization day 2, the anemia did not show improvement. A second angiography revealed extravascular leakage at other intercostal arteries; subsequently, second TAE was performed. Because her anemia did not improve, third angi-ography was performed on hospitalization day 4.
Extravascular leakage of contrast medium was then identified at the supreme intercostal artery and anterior intercostal branch of the internal thoracic artery; therefore, we performed third TAE, following which the bleeding was controlled; however, APTT prolongation did not improve. Considering the rebleeding risk, we administered plasma-derived FVIIa and FX (Byclot^®) and prednisone for immunosuppression therapy and plasma exchange to remove the inhibitors. Thereafter, APTT was shortened and VIII activity increased.
Uncontrolled bleeding is fatal in most AHA patients. In this case, TAE resulted in he-mostasis against arterial bleeding, and subsequent multidisciplinary medical treatment was lifesaving.