A case of acquired hemophilia diagnosed by intrathoracic hematoma that occurred after pneumothorax surgery

Abstract

We report a case of severe chest wall muscular hemorrhage caused by acquired hemophilia A. The patient was a 74-year-old man. He was urgently hospitalized with a diagnosis of right secondary pneumothorax caused by emphysematous cyst and began chest drainage. Blood sampling at admission showed that PT was normal, but APTT was prolonged to 61.2 seconds. A large volume of air leakage persisted after the start of treatment, and we performed VATS bullectomy on the 8th day of hospitalization. On post-operative day (POD) 1, after we removed the drainage tube, a hematoma in the muscle layer of the chest wall appeared just below the removal wound. Blood transfusion and chest band compression were performed to control expansion of the hematoma. Blood tests detected decreased blood coagulation factor VIII activity and factor VIII inhibitor, and the patient was diagnosed with acquired hemophilia A. We subsequently started steroid treatment. Two weeks later, normalization of APTT and factor VIII activity was observed, and disappearance of the inhibitor was confirmed three weeks after the start of administration. Although acquired hemophilia A is a relatively rare disease, there are increasing reports of it being diagnosed as a result of post-operative bleeding complications, and there are cases in which the disease progresses to a fatal outcome. Since early diagnosis and therapeutic intervention affect the prognosis, it is a disease that surgeons should have knowledge of.