B53-20 Autoimmune Anarchy: Refractory Evans Syndrome and Multiorgan Failure

Abstract Introduction Evans syndrome is a rare autoimmune disorder characterized by the coexistence of two or more cytopenias, most commonly autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Management is often complex and requires multiple lines of immunosuppressive therapy. We present the case of refractory Evans syndrome complicated by severe transfusional iron overload resulting in multiorgan failure and death. Case A 39-year-old with a history of refractory Evans syndrome secondary to AIHA and ITP presented with mouth pain. Her Evans syndrome was diagnosed at age five. Through her extensive treatment course, she recieved multiple therapies including intravenous immunoglobulin (IVIG), corticosteroids, rituximab, romiplostim, eltrombopag, fostamatinib, avatrombopag, cyclosporine, and sirolimus. Despite treatment, she experienced severe anemia and thrombocytopenia, ultimately undergoing splenectomy in 2023. On this admission, laboratory evaluation revealed anemia and thrombocytopenia. Dexamethasone, sirolimus, and romiplostim were initiated, leading to resolution of thrombocytopenia, but her severe anemia persisted. Over a 90-day hospitalization, her hemoglobin ranged from 3.8 - 9.0 g/dL, requiring more than 70 red blood cell transfusions. She was subsequently admitted to the intensive care unit (ICU) for hypotension and hypoxia secondary to profound anemia. Her labs were notable for a total bilirubin 84 mg/dL, direct bilirubin 30 mg/dL, and ferritin 33,000 ng/mL. Magnetic resonance imaging (MRI) revealed marked hepatic iron overload. Despite supportive measures, she developed progressive liver failure, renal failure, encephalopathy, and respiratory failure. After discussions with the patient and her family, care was transitioned to comfort-focused measures, and she passed peacefully. Discussion This case illustrates transfusional iron overload as a cause of multiorgan failure in patients without traditional risk factors such as hemoglobinopathies. In refractory Evans syndrome, chronic transfusion dependence may develop as the disease progresses. While transfusions are lifesaving, cumulative exposure can result in iron toxicity and irreversible organ injury. Transfusional iron overload is well recognized in thalassemia and sickle cell disease but less appreciated in autoimmune cytopenias like Evans syndrome. Consequently, iron accumulation may go undetected until significant dysfunction occurs. Early monitoring with ferritin levels, liver function tests, and imaging (e.g., MRI) may allow detection before irreversible injury develops. This case underscores the importance of proactive iron surveillance and consideration of chelation therapy in patients with chronic transfusion requirements due to autoimmune cytopenias. This abstract is funded by: None