Acquired Factor VIII Deficiency Presenting as Compartment Syndrome
Background: Compartment syndrome of the upper extremity is a surgical emergency that most often presents after an identifiable event, such as a crush injury, a closed fracture, a snake bite, reperfusion after embolectomy, an electrical injury, or a circumferential burn.
Case Presentation: We report an unusual case in which an older woman was diagnosed with acquired factor VIII deficiency after presenting with acute nontraumatic compartment syndrome in her right upper extremity. Emergency medicine, plastic surgery, internal medicine, hematology, and hand therapy specialists worked together to identify the pathology and successfully treat the patient.
Conclusions: An isolated elevated partial thromboplastin time level in a patient who is bleeding should raise suspicions for factor VIII deficiency and immediately trigger further evaluation. Once this diagnosis is suspected, multidisciplinary treatment is indicated for immediate and long-term successful outcomes.
Acquired Hemophilia A
Acquired hemophilia A is an autoimmune disease most often found in older individuals, with a mean age of approximately 70 years.12 It is caused by the spontaneous production of neutralizing immunoglobin autoantibodies that target endogenous FVIII. Many cases are idiopathic; however, up to 50% of cases are associated with underlying autoimmunity, malignancy (especially lymphoproliferative disorders), or pregnancy. It often presents as bleeding that is subcutaneous or in the gastrointestinal system, muscle, retroperitoneal space, or genitourinary system. Unlike congenital hemophilia A, joint bleeding is rare.13
The diagnosis is suspected with an isolated elevated PTT in the absence of other coagulation abnormalities. A 1:1 mixing study will typically show incomplete correction, which suggests the presence of an inhibitor. FVIII activity is reduced, and the FVIII inhibitor is confirmed with the Bethesda assay. Clinically active bleeding is treated with bypassing agents such as recombinant coagulation-activated FVII, activated prothrombin complex concentrates such as anti-inhibitor coagulant complex (FEIBA), or recombinant porcine FVIII.12,14 Not all patients require hemostatic treatment, but close monitoring, education, recognition, and immediate treatment, if needed, are indicated.13 Immunosuppressive therapy (corticosteroids, rituximab, and/or cyclophosphamide) is prescribed to eradicate the antibodies and induce remission.12
Conclusions
An older woman without a preceding trauma was diagnosed with an unusual case of acute compartment syndrome in the forearm. No hematoma was found, but muscle and skin bleeding plus an elevated PTT prompted a hematology workup, and, ultimately, the diagnosis of FVIII inhibitor secondary to acquired hemophilia A.
While a nontraumatic cause of compartment syndrome is rare, it should be considered in differential diagnosis for clinicians who see hand and upper extremity emergencies. An isolated elevated PTT in a patient with a bleed should raise suspicions and trigger immediate further evaluation. Once suspected, multidisciplinary treatment is indicated for immediate and long-term successful outcomes.
Acknowledgments
This manuscript is the result of work supported withresources and the use of facilities at the North Florida/South Georgia Veterans Health System, Gainesville, Florida.
