Snapshot A 25-year-old pregnant woman presents with fatigue and abdominal pain in the emergency room. She reports feeling unwell for the past few days. Every morning, she notices that her urine is tea-colored, which slowly resolves throughout the day. She has a low-grade fever. On physical exam, her skin is jaundiced, her liver edge is palpable, and she has abdominal tenderness to palpation. Her physicians are suspicious of Budd-Chiari syndrome. Introduction Clonal stem cell defect from mutation in hematopoietic stem cell Pathogenesis login to view 6 more bullets "PNH Thrombosis" login to view 4 more bullets Acute hemolysis often triggered by stress, infection, surgery, and other inflammatory states May cause login to view 3 more bullets Presentation Symptoms/physical exam login to view 7 more bullets Evaluation Flow cytometry – most accurate test login to view 1 more bullet Labs login to view 3 more bullets Differential Diagnosis Aplastic anemia Myelodysplasia Treatment Eculizumab login to view 4 more bullets Allogeneic bone marrow transplant Supportive therapy login to view 2 more bullets Prognosis, Prevention, and Complications Prognosis login to view 1 more bullet Complications login to view 4 more bullets