A 76‐year‐old man presented with 10 days of persistent hematuria and lower abdominal pain. He also complained of fatigue and dyspnea on exertion. He denied dysuria, flank pain, or recent trauma. His vital signs were normal with the exception of tachypnea. He had conjunctival pallor; the cardiac and pulmonary examinations were normal, and there was no jugular venous distention or edema of the extremities. The phalanges of both upper extremities revealed chronic deformities and joint destruction. His abdominal examination was normal and there was no blood in the stool. His hemoglobin was 7 g/dL; there was no evidence of hemolysis on the peripheral smear. Based on the history of hematuria, a cystoscopy was performed, which revealed blood seeping from the wall of the bladder. Coagulation studies revealed a prothrombin lime of 109 and a factor VIII level that was undetectable and a factor VIII inhibitor level of 60. He had no history of bleeding episodes in the past.
Determining the cause of bleeding is a time‐sensitive issue; failure to identify the cause can result in fatal outcomes. Although hospitalists are trained to identify the source of Ihe hemorrhage, it is equally important to determine that the patient's natural coagulation support system is intact while pursuing the cause. Hemophilia, including factor VIII and factor IX deficiencies, causes a prolonged activated partial thromboplastin time (aPTT), although factor VII deficiency causes an elevated prothrombin time (PT). Congenital hemophilia is the result of a deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B). Although congenital hemophilias are rare in the adult population, the hospitalist should be aware of acquired hemophilia as a cause of sudden, unexplained bleeding. Acquired hemophilia is an autoimmune disorder that occurs when autoantibodies are formed against 1 or more of the factors in the clotting cascade. This usually occurs following an antigenic exposure that prompts the excessive antibody production. By antigenic mimicry, the antibodies target the clotting cascade, disabling the activity of the clotting factor. Usually, this autoantibody is to factor VIII (acquired hemophilia A) and typically arises in older individuals. In half the patients that present with acquired hemophilia, there is an additional process involved; such as a malignancy or rheumatologic disorder. The hospitalist should exercise due diligence in excluding these underlying diseases.
As opposed to congenital hemophilia which presents with hemarthroses; acquired hemophilia presents with bleeding in the mucosa, gastrointestinal system, and urinary tract. Treatment most importantly consists of discontinuation of the bleeding and removal of the antibodies (inhibitor) to the clotting factor. Bleeding can be controlled with Novo‐7 or FEIBA. Prednisone with or without the use of cyclophosphamide will eradicate the inhibitor.
J. Lamberth, none.