Open AccessAcquired hemophilia A: Presenting as a compartment syndrome
Author(s) -
S Sathe
Publication year - 2021
Publication title -
journal of clinical case studies reviews and reports
Language(s) - English
Resource type - Journals
ISSN - 2634-680X
DOI - 10.47363/jccsr/2021(3)193
Subject(s) - medicine , bleed , recombinant factor viia , partial thromboplastin time , coagulation , surgery , immunosuppression , mucocutaneous zone , clotting factor , disease
Acquired hemophilia A (AHA) is a condition caused by autoantibodies (mostly immunoglobulin G) against clotting factor VIII in patients with no prior bleeding disorders. It is usually associated with other autoimmune conditions. Subcutaneous bleed, gastrointestinal bleed, deep muscle bleeds, and mucocutaneous bleedings are some of the common manifestations. Understanding the management principles (control the bleeding, eradication of the inhibitors by the immunosuppression) is prudent as late and inaccurate diagnosis can make it fatal. Here, we present a patient with compartment syndrome due to bleeding into the muscles. Laboratory study was remarkable for prolonged active partial thromboplastin time (aPTT) of 62.9 seconds (normal30-40 seconds), factor VIII (FVIII) activity around 2% (normal is 50%-150%), and high inhibitors levels. Due to concern for AHA, she was treated with steroids and NovoSeven (coagulation factor VIIa, recombinant), leading to stabilization of bleeding. Subsequently, the patient was diagnosed with AHA