Forearm compartment syndrome due to acquired hemophilia that required massive blood transfusions after fasciotomy: A case report

BACKGROUND: Acquired hemophilia is rare. In some cases, the bleeding in muscle causes compartment syndrome. However, it is not clear whether fasciotomy should be performed for the compartment syndrome caused by acquired hemophilia because of the risk of bleeding and the unknown functional results. CASE SUMMARY: A 75-year-old woman was admitted with severe pain of the right forearm with no preceding traumatic event. The right forearm was obviously swollen, and stretch pain was observed. Subcutaneous hematomas were suspected in various parts of the body. Compartment pressure was 110 mmHg on the volar side. Activated partial thromboplastin time (aPTT) was prolonged to 54.9 s. Fasciotomy was performed, and hematoma was observed in the volar compartment. Postoperative laboratory examinations revealed a low level of factor VIII (FVIII) activity (12.5%) and a high level of FVIII inhibitor (15.2 bethesda units/mL). Acquired hemophilia A was diagnosed. Though recombinant clotting factors were administered, transfusion of red blood cells reached 46 units (140 mL/unit). Hemostasis was achieved 9 d after fasciotomy. The total cost of the clotting factor concentrates administered reached 28834600 yen. With prednisolone, FVIII activity and aPTT recovered gradually. Final function of the hand was good in the index finger and excellent in the others. CONCLUSION: Fasciotomy resulted in good function of the hand in a case of non-traumatic compartment syndrome caused by acquired hemophilia, but life-threatening bleeding occurred, and the cost of clotting factor treatment was high. Preparation of sufficient blood transfusion, preoperative administration of recombinant activated clotting factor VII, and prompt fasciotomy could be ideal for such cases.