A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review

Hemophilia is a bleeding diathesis that is most commonly congenital and causes a tendency for significant bleeding during procedures and often manifests as hemarthrosis. However, more rarely, hemophilia can be acquired. Our paper focuses on acquired hemophilia A (AHA), which is caused by the development of an autoantibody (an inhibitor) to factor VIII. A 61-year-old man with a past medical history of type II diabetes mellitus, hypertension, hyperlipidemia, hypothyroidism, and obstructive sleep apnea presented to the emergency department with severe right lower extremity pain and swelling of 2-day duration. He was found to have compartment syndrome and underwent emergent fasciotomy of his right leg. After surgery he still had significant bleeding, despite transfusions and administration of fresh frozen plasma (FFP) by the surgical team. He was later diagnosed with AHA, but was not adequately responsive to factor VII, factor VIII, steroids nor rituxan and unfortunately had his right lower extremity amputated. He had a prolonged hospital course, which included Streptococcus bovis bacteremia and a code stroke for which head computed tomography (CT) showed probable metastasis. It was acknowledged he had probable metastatic colon cancer, which was not confirmed as the patient transitioned to hospice care. Rather than hemarthrosis, patients with AHA tend to have bleeding in soft tissue or the gastrointestinal tract. AHA can have underlying causes, such as malignancy. AHA associated with malignancy is associated with poorer outcomes and tends to improve with treatment of the underlying malignancy. Therefore, it is important to quickly identify these patients and screen them for underlying etiologies.