REFRACTORY HEMORRHAGIC SHOCK SECONDARY TO ACQUIRED HEMOPHILIA A

Abstract

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Hemorrhagic shock is a potentially lethal condition encountered in the intensive care unit. Recognition of the bleeding source and coagulopathic comorbidities is imperative. Acquired factor VIII inhibitor is a rare but devastating cause of hemorrhagic shock. CASE PRESENTATION: An elderly woman who recently sustained a humeral fracture complicated by pain and excessive Ibuprofen use was admitted with hematemesis. She had a bleeding gastric ulcer which was endoscopically treated without complication. She required 6 units of blood and was discharged home. A week later, she was re-admitted with a syncopal episode. Her hemoglobin was 6.1 g/dL. Computed tomography (CT) showed retroperitoneal (RP) bleeding. She was transferred to our tertiary hospital and was found to be in shock. Blood work revealed prolonged activated prothrombin time and normal prothrombin time. She had not received heparin products in over 3 weeks. Mixing studies did not correct the defect, suggesting the presence of an inhibitor. Testing of factor VIII showed reduced activity at 8%. A Bethesda assay confirmed very high titers of factor VIII inhibitor (1:64). She was started on massive transfusion protocol and Factor VIII Inhibitor Bypassing Activity (FEIBA) infusion. Patient had 2 cardiac arrests followed by return of spontaneous circulation (ROSC). Repeat CT angiography showed enlarging RP hematoma. Interventional radiology angio-embolized multiple arteries with endovascular coils; however, a clear bleeding source could not be identified. The patient had two more cardiac arrests and progressed to multi-organ system failure. After a family meeting, she was transitioned to comfort care and passed away. DISCUSSION: Factor VIII inhibitors are the most common acquired inhibitors of coagulation. Acquired hemophilia A is a rare condition with poorly understood etiology often associated with pregnancy, rheumatologic diseases, malignancies or drug reactions. Our patient had a normal coagulation profile prior to her gastrointestinal bleed requiring transfusion of blood products. While rare, there have been reports of developing an acquired inhibitor of coagulation after administration of blood products or derivatives. Even with aggressive treatment regimens, mortality remains high, especially with elevated inhibitor titers. CONCLUSIONS: Acquired factor inhibitor is a rare but devastating cause of hemorrhagic shock. Providers should test for an acquired inhibitor of coagulation in patients with spontaneous bleeding in the absence of known trauma or coagulopathy. Despite high mortality rates, early diagnosis with prompt initiation of factor directed therapy is essential. Reference #1: Kruse-Jarres R, Kempton CL, Baudo F, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92:695–705. https://doi.org/10.1002/ajh.24777 Reference #2: Kessler, C. M. and Knöbl, P. (2015), Acquired haemophilia: an overview for clinical practice. Eur J Haematol, 95: 36-44. https://doi.org/10.1111/ejh.12689 Reference #3: Kajitani, M. , Ozdemir, A. , Aguinaga, M. , Jazieh, A. , Flick, J. T. and Antakli, T. (2000), Severe Hemorrhagic Complication Due to Acquired Factor V Inhibitor After Single Exposure to Bovine Thrombin Product. Journal of Cardiac Surgery, 15: 378-382. https://doi.org/10.1111/j.1540-8191.2000.tb01296.x DISCLOSURES: No relevant relationships by Salim Daouk, source=Web Response No relevant relationships by Amanda Moyer, source=Web Response No relevant relationships by Ibrahim Raphael, source=Web Response