SHM Converge Scientific Abstract ePoster Gallery-Presentation and Management of Acquired Factor Viii Deficiency

Pdf Summary

This document reviews <strong>acquired hemophilia A (AHA)</strong>, a <strong>rare autoimmune bleeding disorder</strong> affecting about <strong>1–2 people per million</strong>. In AHA, the body develops <strong>autoantibodies against factor VIII (FVIII)</strong>, which blocks the intrinsic clotting pathway and can cause <strong>severe, potentially life-threatening bleeding</strong>.

A featured case describes a <strong>59-year-old woman</strong> with irritable bowel syndrome and recent <strong>shingles</strong> who developed <strong>painful ecchymoses, swelling, and subcutaneous nodules</strong> in her arms, initially raising concern for <strong>deep vein thrombosis</strong>. Her evaluation showed a broad differential, including <strong>coagulopathy, vasculitis, rheumatologic disease, and malignancy</strong>. Imaging and autoimmune testing were not diagnostic, and biopsy revealed <strong>localized hematomas and nonspecific myositis</strong>.

Laboratory findings supported AHA:  
- <strong>Severe FVIII deficiency</strong>  
- <strong>Prolonged aPTT</strong> that did <strong>not correct on mixing study</strong>  
- <strong>Bethesda assay positive</strong> for an FVIII inhibitor (**5.6 Bethesda units**)

She was treated with <strong>recombinant FVIII</strong> and <strong>prednisone</strong>. Additional workup for <strong>autoimmune disease and occult malignancy</strong> was negative.

The document emphasizes that AHA should be suspected in <strong>unexplained bleeding or bruising</strong>, especially when accompanied by an <strong>isolated prolonged aPTT</strong> and failure to correct with mixing studies. Common triggers include <strong>viral infections, autoimmune disorders, malignancy, medications, and postpartum state</strong>.

Overall, the case highlights the importance of a <strong>broad, stepwise diagnostic approach</strong> to unusual bleeding presentations and notes that <strong>steroids and cytotoxic immunosuppressive agents appear similarly effective</strong>, though <strong>relapse occurs in about 20%</strong> of cases.

Asset Subtitle

Joseph A. Menand

Meta Tag

Author List Joseph A. Menand, Rohit Nathani, Susan Lin

Category Clinical Vignettes

Concept Acquired Hemophilia A

Concept Partial Thromboplastin Time

Concept Factor VIII Deficiency

Concept Factor VIII Inhibitor

Concept Bruising

Distinguished Non-Finalist

Presenter Organization Mount Sinai Morningside/West

Presenting Author Joseph A. Menand

Track Adult

Keywords

acquired hemophilia A

autoimmune bleeding disorder

factor VIII inhibitor

prolonged aPTT

mixing study

Bethesda assay

unexplained bruising

ecchymoses

recombinant FVIII

prednisone

Acquired Hemophilia A

Partial Thromboplastin Time

Factor VIII Deficiency

Factor VIII Inhibitor

Bruising