SHM Converge Scientific Abstract ePoster Gallery-Not All Antibodies Are Protective- Some Can Cause a Bloody Mess!

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This case describes a rare, life-threatening bleeding disorder, acquired hemophilia A (AHA), caused by autoantibodies against factor VIII. Unlike congenital hemophilia, which often causes hemarthroses, AHA typically presents with mucosal, gastrointestinal, or soft tissue bleeding.<br /><br />A 78-year-old woman with atrial fibrillation and a recent GI bleed presented with severe left hip pain and profound anemia (hemoglobin 5.3 g/dL). Imaging showed a left gluteus medius hematoma, and she also had melena from a Dieulafoy lesion and hematuria. Laboratory evaluation revealed an isolated prolonged aPTT (78 seconds) with normal PT and incomplete correction on mixing study, suggesting an inhibitor. Further testing confirmed factor VIII activity of 3% with a factor VIII inhibitor of 17.5 Bethesda units, establishing the diagnosis of acquired hemophilia A.<br /><br />She was initially treated with FEIBA, cyclophosphamide, and prednisone. Her hematuria and GI bleeding improved, but on hospital day 14 her hemoglobin fell again, and repeat CT showed expansion of the left psoas and iliacus hematoma. Therapy was escalated to alternating FEIBA and recombinant factor VIIa, and rituximab was substituted for cyclophosphamide. Despite these measures, she developed progressive decline, multi-organ failure, and ultimately transitioned to comfort care before passing away.<br /><br />The case highlights the importance of recognizing AHA in patients with unexplained bleeding, isolated prolonged aPTT, and normal PT. Severe cases require both hemostatic control with bypassing agents and immunosuppression to suppress factor VIII inhibitors. Prompt diagnosis and treatment are critical, as mortality remains significant.

Asset Subtitle

Shivani Shah

Meta Tag

Author List Deep Shah, Maitreyee Rai, Ryan Sweeney, Shivani Shah

Category Clinical Vignettes

Concept Acquired Hemophilia A

Concept Partial Thromboplastin Time

Concept Mixing Study

Concept Bypassing Agent

Concept Immunosuppressive Therapy

Distinguished Non-Finalist

Presenter Organization Allegheny General Hospital

Presenting Author Shivani Shah

Track Adult

Keywords

acquired hemophilia A

factor VIII inhibitor

prolonged aPTT

soft tissue hematoma

gastrointestinal bleeding

hematuria

FEIBA

rituximab

bypassing agents

immunosuppression

Acquired Hemophilia A

Partial Thromboplastin Time

Mixing Study

Bypassing Agent

Immunosuppressive Therapy