Acute Compartment Syndrome of the Upper Extremity in Acquired Hemophilia A: A Case Report and Literature Review.

Case: Acute compartment syndrome (ACS) with acquired hemophilia A (AHA) is rare and has no established treatment strategy. A 64-year-old woman presented with a giant hematoma in the rectus abdominis. Laboratory findings included decreased hemoglobin and increased activated partial thromboplastin time. Arterial embolization was performed for hemostasis. After catheter removal, she developed severe arm pain and numbness with blistering. Fasciotomy was performed to decrease intracompartmental pressure. Laboratory investigations revealed decreased factor VIII (FVIII) activity and increased FVIII inhibitor. AHA was diagnosed and treated with immunosuppressive and FVIII inhibitor-bypassing agents.

Conclusions: Fasciotomy should be performed promptly if ACS with AHA is suspected.