A case of acquired hemophilia A with massive hemothorax

Abstract

Acquired hemophilia A (AHA) is an uncommon but potentially life-threatening hemorrhagic disorder caused by the development of an inhibitor against coagulation factor VIII (FVIII). AHA is very rare, affecting approximately 1 in 1 million individuals. However, the incidence may actually be higher, because diagnosis is difficult and the disease can be overlooked. We report a case of an 80-year-old man who presented with sudden onset of severe hemothorax. The patient was diagnosed with presumed AHA based on acute onset of bleeding symptoms and unexplained isolated prolonged activated partial thromboplastin time. Diagnosis was definitely established by demonstrating a decrease in FVIII activity, presence of FVIII inhibitor activity, and normal von Willebrand factor. The patient was successfully treated with recombinant activated coagulation factor VII and transcatheter artery embolization of the intercostal arteries.

Acquired hemophilia A as a cause of recurrent bleeding into the pleural cavity – and literature review

“…Bleeding into the pleural cavity caused by acquired hemophilia is very uncommon (approx. 1% of cases), but is usually potentially fatal and may appear after surgical procedures performed within the area of the chest [ 3 , 5 , 16 ]. Bleeding within the area of the chest had been described in women with acquired hemophilia after esophageal resection due to cancer [ 16 ].…”

“…Both operations were performed via posterolateral thoracotomy. Embolization of the bleeding vessels and videothoracoscopic procedures due to similar indications have also been described [ 3 ]. In acquired hemophilia, the bleeding is often recurrent in nature and is very difficult to stop with the use of surgical methods [ 16 , 21 ].…”

“…It is equally common in both sexes and usually appears at old age. This potentially fatal disorder is caused by the activity of autoantibodies impairing the function of blood coagulation factors, mainly factor VIII (acquired hemophilia A) [ 1 – 3 ]. In approx.…”

“…The bleeding events may be spontaneous or follow a surgical procedure or injury [ 5 ]. They include hemorrhages into the hypodermis, digestive tract, genitourinary system, retroperitoneal space, as well as into the respiratory system and pleural cavity [ 1 , 3 ]. Mortalities have been reported as early as in the first week after the occurrence of bleeding from the digestive tract and lungs.…”

“…Therefore, spreading the knowledge about this disease may further contribute to lowering the percentage of deaths. The characteristic features of acquired hemophilia include isolated prolonged APTT, low activity of the coagulation factor targeted by the antibodies, and the presence of antibody titer [ 2 , 3 , 11 ]. The present study discusses a case of a young man with recurrent, massive bleeding into the pleural cavity caused by acquired hemophilia A and presents an analysis of literature devoted to acquired hemophilia.…”