Severe relapse of Evans syndrome in an adult patient with treatment resistance and fatal outcome: A case report

Evans syndrome is an uncommon autoimmune disorder characterized by autoimmune hemolytic anemia and immune thrombocytopenia. It can follow a relapsing course in adults and may be challenging to treat. A woman in her 50s, diagnosed with Evans syndrome 2 years ago and on 10 mg prednisolone once daily, presented with worsening symptoms, lymphadenopathy, and abnormal uterine bleeding. Lymph node biopsies revealed caseating and noncaseating granulomas, and infections and systemic autoimmune diseases were ruled out. She required intensive care unit admission and treatment with high-dose corticosteroids and rituximab. Plasmapheresis was attempted as the last-line therapy; however, it resulted in hemodynamic instability. This case reflects the clinical challenges involved in managing relapsed Evans syndrome in the absence of a clear secondary cause. The presence of granulomatous inflammation raises questions about lesser-known immune features of the disease. The use of plasmapheresis in unstable patients should be approached with caution.