Orthopedic manifestations of acquired hemophilia: a case report on uncontrolled bleeding and progressive swelling post-phlebotomy- should fasciotomy be considered?

backgroundAcquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factors, often resulting in spontaneous bleeding or bleeding after minor trauma, potentially leading to Acute Compartment Syndrome (ACS). Diagnosing and managing AHA-induced ACS is challenging due to its rarity and the risks associated with fasciotomy in bleeding disorders. In low- and middle-income countries (LMICs), ACS management is further complicated by the lack of intra-compartmental pressure monitoring devices, necessitating reliance on clinical symptoms. CASE PRESENTATION: A 36-year-old woman presented to the emergency department with progressive swelling of the left antecubital area after phlebotomy with no trauma or bleeding disorder history. After hospitalization a venous color Doppler ultrasound showed superficial vein thrombosis, and the patient was discharged and referred to the orthopedic service for follow-up of clinical symptoms. Returned to the hospital after approximately six hours due to the development of generalized weakness and hemodynamic instability. Diagnostic challenges arose as initial laboratory results suggested possible acute leukemia due to leukocytosis and thrombocytosis. Her condition deteriorated rapidly with wrist drop which drew increased attention and necessitating a fasciotomy due to suspected ACS, revealing profuse bleeding. Subsequent tests revealed low factor VIII levels and a high factor VIII inhibitor, confirming AHA. Treatment included empirically activated factor VII, prednisolone, cyclophosphamide, and rituximab, resulting in gradual improvement but residual wrist drop.

conclusionsThis case underscores the importance of considering AHA in patients with ACS-like symptoms and unexplained prolonged activated Partial Thromboplastin Time (aPTT), even without bleeding history. It also highlights the need to investigate any unexplained aPTT prolongation before surgical interventions. Collaboration between orthopedic surgeons and hematologists is essential for optimizing outcomes. Approaching to algorithms for non-traumatic ACS in AHA is an important step. Prompt intervention, patient education, and tailored treatment strategies are crucial for preventing severe complications and optimizing patient outcomes.