Anahat Kaur MD¹, Bhanujit Dwivedi MBBS^2,, Tejasvi Dwivedi MBBS³, Rubina Sharma MD⁴, Angad Singh MD⁵

Evans syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential presence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Secondary Evans syndrome refers to cases in which these autoimmune manifestations occur in the setting of an underlying condition or trigger. We report the case of a 36 year old male with no significant past medical history who presented with symptoms of easy bruising, and mucocutaneous bleeding. Laboratory investigations revealed severe thrombocytopenia and hemolytic anemia, consistent with the diagnosis of Evans syndrome. Further evaluation demonstrated the presence of triple positive anti-phospholipid antibodies, including lupus anticoagulant, anticardiolipin antibodies, and anti-beta-2 glycoprotein antibodies. Patient was initially treated with steroids and IVIG but had refractory ITP for several weeks requiring rituximab (four weekly doses) and fostamatinib before eventual improvement and stabilization of platelet count. Given the rarity of secondary Evans syndrome with positive anti-phospholipid antibodies, there is a lack of robust evidence-based treatment guidelines. Our case highlights the complexity and challenges encountered in managing this condition.

Secondary Evans, Anti-phospholipid antibodies, Refractory ITP