Conflict of Interest: None declared
Hemophilia A is a bleeding disorder caused by defective production of factor VIII. The main concern associated with the disease is bleeding, especially after trauma and surgeries. Factor VIII replacement therapy is associated with substantial decrease of bleeding events during surgery. However, there have been a number of reports of thromboemblic events in this situation. The present report describes a case of moderate hemophilia A in which splenectomy did lead to pulmonary embolism and subsequent death. The patient was a 25-year-old man with hemophilia A admitted after a car accident Inhibitors,research,lifescience,medical and trauma to left lower chest and abdomen. He received
factor VIII concentrates for replacement therapy. He was hemodynamically stable on the first day, but on the Inhibitors,research,lifescience,medical second day his hemoglobin declined and he showed signs of abdominal tenderness. He, therefore, was subjected to laparatomy and splenectomy. After the operation, he suddenly developed dyspnea and decline in blood pressure, and death afterwards. Inhibitors,research,lifescience,medical Autopsy of the patient revealed massive pulmonary thromboembolism. The symptoms and outcome of the present case indicate that although pulmonary thromboembolism in the early postoperative period in patients with hemophilia A undergoing splenectomy and receiving factor VIII concentrate for replacement
is rare, it should not be MGCD0103 datasheet assumed a far-fetched event, and prophylactic measures to prevent thromboemboly must be considered. Key Words: Hemophilia A, pulmonary thromboembolism, Inhibitors,research,lifescience,medical splenecetomy, factor VIII Introduction Hemophilia A is a congenital X chromosome–linked hemorrhagic disorder caused by a deficit or defective functioning of clotting factor VIII. The incidence of haemophilia is around one in every 5000 males.1 Hemophilia is classified clinically into three categories Inhibitors,research,lifescience,medical on the basis of severity of factor VIII deficiency including severe, moderate and mild. Severe hemophilia (factor VIII level <1 Iu/dl or <1%
factor VIII activity) is manifested by repeated and severe hemarthrosis or hemorrhage with or without trauma. Moderate hemophilia (factor VIII level 1 to 5 Iu/dl) is associated with less frequent and less severe hemorrhage, and affected patients have occasional hematoma and hemarthrosis, which usually but not always, are associated with known trauma. Mild hemophilia is defined by factor VIII levels between 5 to 40 IU Iu/dl. Spontaneous bleeding is rare in mild hemophilia, and bleeding associated Edoxaban with mild haemophilia most frequently occurs during surgery or following trauma.2,3 Currently, treatment of serious bleeding in all subtypes of hemophilia A is facilitated by the introduction of various factor VIII concentrates for replacement therapy. The amount of factor VIII must be enough to ensure that its blood level does not fall to <30 to 50 IU/dl for any length of time. Maintenance doses are usually given every 8 to 12 hours.