S4802 An Autoimmune Triple Threat: Evans Syndrome With APLA Causing Portal Vein Thrombosis and Recurrent GI Bleeding

Introduction: Evans syndrome (ES) is a rare autoimmune disorder combining autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Its coexistence with antiphospholipid antibodies (APLA) is uncommon but clinically significant. We present a 47-year-old woman with ES and APLA who developed chronic portal vein thrombosis (PVT) with cirrhosis, leading to recurrent variceal gastrointestinal (GI) bleeding and sepsis. This case highlights diagnostic and therapeutic complexities involving hematology and hepatology. Case Description/Methods: In 2022, the patient presented with fatigue, jaundice, and melena. Labs showed Hb 6.2 g/dL, platelets 8 × 10³/μL, elevated LDH (980 U/L), undetectable haptoglobin, and a positive direct antiglobulin test confirming warm AIHA. ANA titer was 1:640 (speckled), and lupus anticoagulant was positive. Imaging revealed a cirrhotic-appearing liver, chronic PVT with cavernous transformation, and splenomegaly. Endoscopy showed large esophageal varices, which were banded. Bone marrow biopsy showed ∼70% hypercellularity, erythroid hyperplasia, and increased megakaryocytes—consistent with peripheral destruction. APLA profile showed high-titer anticardiolipin IgG (∼80 GPL) and β2-glycoprotein I IgG (∼60 SGU) on 2 occasions, confirming APS. High-dose corticosteroids and IVIG induced transient remission (Hb 10, platelets 150 × 10³/μL). Warfarin was started once bleeding was controlled. Between 2022–2024, she had relapses of ES and variceal bleeds. One ITP flare (platelets 50 × 10³/μL) responded to rituximab. Two variceal bleeds in 2023 required urgent banding, PPIs, and octreotide. She developed septic shock twice—SBP in 2023 and pneumonia in 2024—both complicated by DIC and cytopenias, needing intensive care unit care, antibiotics, transfusions, and holding immunosuppression. By 2025, she achieved stable remission (Hb ∼11, platelets ∼100 × 10³/μL) on low-dose steroids and IVIG. PVT remained stable; nonselective β-blockers were added. Discussion: This case demonstrates an unusual overlap of autoimmune cytopenias and thrombosis manifesting as portal hypertension. APS-related PVT can cause variceal bleeding and hypersplenism-related cytopenias. ES increased bleeding and infection risks. Anticoagulation was carefully timed around bleeds, in line with individualized PVT guidelines. This case underscores that thrombocytopenia in cirrhosis isn’t always due to hypersplenism and highlights the need to consider APS-related splanchnic thromboses. Collaborative, tailored care led to disease stability.