Acquired Hemophilia A With Post-Phlebotomy Compartment Syndrome

Area of Science:

• Hematology
• Internal Medicine
• Clinical Case Reports

Background:

• Acquired hemophilia A (AHA) is a rare autoimmune disorder characterized by the development of autoantibodies against coagulation factor VIII.
• It presents with severe spontaneous bleeding, often without a prior history of bleeding disorders.
• Diagnosis can be challenging due to its rarity and varied clinical manifestations.

Purpose of the Study:

• To highlight an atypical presentation of acquired hemophilia A.
• To emphasize the importance of considering AHA in patients with unexplained bleeding and isolated activated partial thromboplastin time prolongation.
• To underscore the role of factor VIII inhibitors in refractory bleeding, even in non-classical scenarios.

Main Methods:

• Case report presentation.
• Review of clinical presentation, diagnostic workup, and management.
• Literature review on atypical presentations of acquired hemophilia A.

Main Results:

• The case presented with compartment syndrome following phlebotomy, a rare initial manifestation of AHA.
• Refractory bleeding was attributed to the presence of factor VIII inhibitors.
• Prompt diagnosis and management led to favorable outcomes.

Conclusions:

• Acquired hemophilia A should be suspected in unexplained bleeding, particularly with isolated activated partial thromboplastin time prolongation, irrespective of severity.
• Compartment syndrome post-phlebotomy can be an unusual presentation of AHA.
• High clinical suspicion is paramount for timely diagnosis and effective management of factor VIII inhibitor-related bleeding.