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Acute abdomen: an atypical presentation of acquired hemophilia

Muhamad Rafiqi Hehsan.




Abstract

Acquired hemophilia, a rare autoimmune disease characterized by the formation of autoantibodies against clotting factors, brings particular challenges in diagnosis and management. We present the unfortunate case of a 66-year-old male with no prior history of bleeding disorders who presented to the emergency department with symptoms of sudden-onset acute abdomen and hypovolemic shock. Upon admission, a physical examination revealed signs of peritonitis, prompting urgent imaging studies. Abdominal computed tomography (CT) scans revealed extensive intraperitoneal haemorrhage with no apparent source. Laboratory investigations revealed a significant drop in hemoglobin levels, prolonged activated partial thromboplastin time (aPTT), and a zero level of factor VIII, establishing the diagnosis of acquired hemophilia. While surgery had been scheduled, the patient developed respiratory distress that necessitated intubation. He was treated with bypassing agents, including recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC). The massive transfusion protocol was also activated, and the patient received blood products and packed red blood cells as well. Hemodialysis treatment was needed due to worsening renal function caused by an acute kidney injury. Unfortunately, the patient's hemodynamics deteriorated during dialysis, requiring double inotropic support until ventricular tachycardia and asystole developed. CPR commenced for 30 minutes; however, he succumbed to death. This case underscores the importance of considering acquired hemophilia in the differential diagnosis of acute abdomen, particularly when encountering unexplained intraperitoneal hemorrhage. The rarity of acquired hemophilia and its potential to masquerade as other acute abdominal emergencies necessitate a high index of suspicion for timely diagnosis and intervention.

Key words: Acute abdomen, hemophilia, bleeding, transfusion






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