Neurologic complications of Evans syndrome

Evans syndrome is a rare autoimmune disorder characterised by concurrent or sequential autoimmune haemolytic anaemia and immune thrombocytopenia, often posing significant diagnostic and therapeutic challenges due to its relapsing nature and variable response to treatment. We report a patient with severe Evans syndrome who developed status epilepticus in the setting of intracranial haemorrhage secondary to profound treatment-refractory thrombocytopenia, illustrating the potential for life-threatening neurological complications when cytopenias are uncontrolled. Despite limited improvement with first-line therapies, the patient experienced haematologic recovery and clinical stabilisation following initiation of avatrombopag, a thrombopoietin receptor agonist. This case highlights the importance of early recognition of treatment refractoriness and supports the emerging role of avatrombopag as a valuable therapeutic option for persistent thrombocytopenia in Evans syndrome.