SHM Converge Scientific Abstract ePoster Gallery-Sometimes When You Hear Hooves, Consider Zebras

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This case report describes a 70-year-old woman with COPD who presented with sudden, unexplained bruising and swelling of the right arm without trauma. She had recently been hospitalized for chest pain, but that workup was negative. Laboratory testing showed isolated, markedly prolonged aPTT with normal INR and platelets, and mixing studies failed to correct after incubation, suggesting an inhibitor rather than a factor deficiency. Factor VIII activity was severely reduced at 2%, and the autoimmune workup was negative, supporting a diagnosis of acquired hemophilia A (AHA), a rare disorder caused by autoantibodies against factor VIII.<br /><br />The report emphasizes that AHA often presents in older adults without personal or family history of bleeding. Unlike congenital hemophilia, it typically causes spontaneous skin, soft tissue, muscle, and mucosal bleeding rather than hemarthroses. Common associated conditions include autoimmune disease, malignancy, pregnancy, medications, and sometimes no identifiable cause.<br /><br />Initial treatment included high-dose prednisone and daily intravenous immunoglobulin. Despite therapy, the patient developed spontaneous gum bleeding on hospital day 2 and later suffered acute hypoxic respiratory failure with PEA arrest and anoxic brain injury on hospital day 3, after which care was withdrawn.<br /><br />The discussion highlights that diagnosis depends on recognizing isolated prolonged aPTT, performing mixing studies, and confirming low factor VIII activity with inhibitor evidence. Management of active bleeding often requires bypassing agents such as recombinant activated factor VII or activated prothrombin complex concentrate, while inhibitor eradication may involve immunosuppressive therapy including steroids, cyclophosphamide, azathioprine, rituximab, or adjunctive IVIG.<br /><br />Overall, the case underscores the importance of considering rare causes of bleeding in patients with new, unexplained hemorrhagic symptoms.

Asset Subtitle

Christine J. Kang

Meta Tag

Author List Chiagozie Nwasuruba, Christine J. Kang

Category Clinical Vignettes

Concept Acquired Hemophilia A

Concept Partial Thromboplastin Time

Concept Mixing Study

Concept Factor VIII Activity

Concept Factor VIII Inhibitor

Distinguished Non-Finalist

Presenter Organization University of Texas Health Science Center at Tyler Internal Medicine Program

Presenting Author Christine J. Kang

Track Adult

Keywords

acquired hemophilia A

prolonged aPTT

factor VIII inhibitor

mixing study

spontaneous bruising

soft tissue bleeding

autoimmune disorder

immunosuppressive therapy

recombinant factor VIIa

older adult bleeding

Acquired Hemophilia A

Partial Thromboplastin Time

Mixing Study

Factor VIII Activity

Factor VIII Inhibitor