Abstract Number: PB0443
Meeting: ISTH 2021 Congress
Background: Acquired Hemophilia A (AHA) is a rare autoimmune bleeding disorder where autoantibodies are directed against clotting factors, most commonly factor VIII. The etiology is poorly understood but it is most commonly associated with pregnancy/postpartum status, malignancy and other autoimmune disorders.
Aims: Case Presentation.
Methods: Our patient is an 84-year-old female with a history of hypertension (HTN), hyperlipidemia (HLD) and left total hip arthroplasty (THA), who presented with progressively worsening pain and bruising to her left posterolateral thigh of 5 weeks duration. Her laboratory studies were significant for Partial Thromboplastin Time (PTT) of 80, hemoglobin of 5.7, platelet count of 650,000 and C reactive protein of 6.3. Computed Tomography of the Pelvis and lower extremity revealed multiple low-attenuation, large hematomas (range: 7-8 cm), which were evidently expanding muscle groups of the posterior thigh, triggering concerns for compartment syndrome.
Acquired hemophilia A is a rare autoimmune disorder with uncertain etiology. One of the major complications resulting from factor VIII assay activity less than 1% is spontaneous hematoma formation. Improvements in Factor VIII assay typically will not be seen until inhibitor levels are below 10 BU. Interestingly, the observed 80% decline in the inhibitor level occurred after only one dose of Rituximab, suggesting a possible synergistic effect between Cyclophosphamide and corticosteroid. There are currently no definitive guidelines governing treatment of AHA.
To cite this abstract in AMA style:Ahmed S, Joseph E, Loughry K. Acquired Hemophilia A Presenting as Spontaneous Intramuscular Hematomas [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/acquired-hemophilia-a-presenting-as-spontaneous-intramuscular-hematomas/. Accessed October 2, 2023.
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