Intractable Lower GI Bleeding due to Acquired Hemophilia A in a Patient With Rectal Cancer

Abstract

Introduction: Acquired hemophilia A (AHA) is a rare bleeding disorder with an estimated incidence of 1-2/million/year. It is caused by production of auto-antibodies which inhibit the coagulant activity of factor VIII. It can be idiopathic or associated with postpartum status, autoimmune disorders, infection and malignancy. We present a case of intractable GI bleeding due to AHA as a paraneoplastic manifestation in a patient with rectal cancer. Case report: An 87 year old male presented with new onset lower GI bleed and easy bruising. A colonoscopy with biopsies diagnosed rectal adenocarcinoma. CT angiogram was then performed due to persistent bleeding and showed extravasation of contrast at the rectal mass. Prolonged activated thromboplastin time (aPTT) of 76 sec was noted, with normal platelets and INR. Further work-up revealed a positive mixing study, severe Factor VIII activity deficiency, and a positive Factor VIII inhibitor. The diagnosis of Acquired Hemophilia A was established. Efforts were made to control the bleeding with recombinant activated factor VII, Factor VIII Inhibitor Bypassing Agent, and Methylprednisolone. The rectum also underwent packing, followed by radiation. Ultimately, due to continued rectal bleeding, he underwent lower anterior resection with end-colostomy. Following surgery, he had surgical wound bleeding requiring multiple abdominal washouts and packing. Hospital course was further complicated by multi-organ failure and family decided to withdraw care.Figure: Angiography shows the gastroduodenal artery before (Fig 1a) and after (Fig 1b) angioembolization.Figure: CT imaging done the day after angioembolization of the gastroduodenal artery showed hypodense non-enhancing areas in the head and uncinate process of pancreas with acute necrotic collections consistent with acute pancreatitis (Fig 2a). The common bile duct (CBD) was not dilated (Fig 2b), however interval CT imaging 3 months later showed significant CBD dilatation (Fig 2c).Figure: EUS (Fig 3a) noted a dilated CBD and distal CBD stricture with no accompanying biliary wall thickening or mass. Contrast hold-up was noted at the distal CBD during ERCP (Fig 3b). Subsequent attempts to cross the CBD stricture during attempted internalization of the percutaneous transhepatic biliary drain were unsuccessful (Fig 3c).Discussion: Acquired hemophilia A is a rare disease that may cause life-threatening GI bleeding. A paraneoplastic phenomenon is reported in approximately 10% of AHA cases. The most common solid cancer associated with AHA is prostate cancer with rare reports of rectal cancer. Time to diagnosis is critical due to the difficulties in treating this disease and its high mortality. Diagnosis is made in a patient with prolonged aPTT not corrected with mixing study and demonstrating: 1) reduced FVIII level and 2) detectable FVIII inhibitor. Treatment includes hemostasis and suppression of inhibitor production. Hemostasis should be attempted with a bypassing agent such as recombinant activated factor VII (rFVIIa) or Factor VIII Inhibitor Bypassing Agent (FEIBA). Immunosuppression options include use of corticosteroids alone or corticosteroids combined with cyclophosphamide. Rituximab has been also been found to be successful as a second line treatment option.Figure: CT angiogram showing luminal extravasation of contrast.Table: Table. Lab Results