A case of Evans syndrome

Abstract

Evans syndrome is an autoimmune disorder described by Robert Evans in 1951 specifying that a link exists between primary thrombocytopenic purpura and acquired haemolytic anaemia. It is a rare autoimmune disorder characterised by the simultaneous or sequential development of autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Approximately, Evans syndrome represent 5-10% of warm autoimmune haemolytic anaemia cases and 2-5% of ITP cases. It is basically a diagnosis of exclusion. We present a 50-year-old female presented with anaemia, jaundice and petechia. Patient indirect bilirubin was raised, Coombs test was positive, lactate dehydrogenase and reticulocyte count were raised. Based on the Coombs positive haemolytic anaemia and thrombocytopenia patient was diagnosed with Evans syndrome. ANA profile was positive for SS-A suggestive of underlying autoimmune disorder. Evans syndrome is difficult to diagnose and treat. Typically, corticosteroids and other immunosuppressive medications are used to manage the syndrome.

Cracking of enigma of Evans: a rare association with Sjogren and systemic lupus erythematosus

“…A 2009 study revealed that in 34 individuals (50%), ES was identified as "primary," while in 50% of cases, it was linked to an underlying condition, predominantly systemic lupus, lymphoproliferative diseases, and common variable immunodeficiency. 10 There are no clinical trials available for ES treatment and indications for starting therapy have not been established by evidence-based studies. Managing ES continues to be a challenging task.…”