(1674) Navigating Hemorrhagic Shock in an Elderly Patient With Newly Diagnosed Acquired Hemophilia A

Introduction: Acquired Hemophilia A (AHA) is a rare autoimmune disorder targeting Factor 8 causing coagulopathy leading to increased risk of life-threatening bleeding. AHA is linked to autoimmune diseases but can be idiopathic, with incidence increasing with age. Early recognition and treatment are crucial to prevent mortality from hemorrhagic shock.

Description: A 75-year-old female with newly diagnosed bullous pemphigoid and history of unexplained anemia requiring blood transfusions, presented to an outside hospital after an unwitnessed fall complicated by splenic laceration and large hematomas of the left iliopsoas muscle and retroperitoneum. Initial hemoglobin (Hb) was 8.6g/dL which decreased to 5.4g/dL. Patient was found to have a prolonged PTT of 90 secs which was thought to be due to a positive lupus anticoagulant. Due to clinical deterioration despite 7-units PRBCs and 1-unit FFP given, decision was made for patient to be transferred to our hospital for Interventional Radiology services. In the ICU, she required pressor support with initials labs showing PT 14.8 (11.8 - 14.8 sec), PTT 90 (24 – 36secs), INR 1.4, vWF 334 (50 - 217 %), fibrinogen 408 (200 - 450 mg/dL) and mixing study with no correction of prolonged clotting time. Despite prothrombin complex concentrate, cryoprecipitate and FFP, there was no clinical improvement with further testing revealing Factor 8 activity of 3% with Factor 8 inhibitor positive and 52.6 Bethesda units ( < 0.6 Bethesda units). Once the diagnosis of AHA was made, she was then managed with recombinant activated factor 7 and steroids with stabilization of her Hb and hematomas on repeat imaging.

Discussion: AHA is a rare condition that can be challenging to identify leading to potential misdiagnosis. This case report highlights a patient with severe anemia and prolonged PTT initially attributed to a positive lupus anticoagulant, who was ultimately diagnosed with AHA. Management is guided using case series and, in this case, bleeding control was found to be more effective with bypassing agents. This case emphasizes the association between autoimmunity and AHA especially in elderly patients presenting with severe anemia and a prolonged PTT, underscoring the importance of maintaining a high clinical suspicion to mitigate morbidity and mortality risks.