Evans Syndrome Triplet in an Infant, a Case Report

Abstract Background Evans syndrome(ES) is a rare autoimmune hematologic disorder defined by autoimmune hemolytic anemia and idiopathic thrombocytopenia and/or neutropenia. Little is known about its epidemiology and prognosis in different age groups. It is usually present in the elderly. The typical presentation of this syndrome involves two hematological lines, most commonly autoimmune hemolytic anemia and immune thrombotic thrombocytopenia. Here, we report a case of an infant diagnosed with Evans syndrome triplet involving the three blood cell lines. Case Presentation: We Present an eight-month-old male who was brought to our department with fever, jaundice, and petechial rash. He had a negative septic workup, and CBC revealed pancytopenia with severe thrombocytopenia, anemia, and neutropenia. Differential diagnoses were excluded by further tests. These included peripheral smears that showed < 10% blast cells, and bone marrow aspiration was normal. A diagnosis of Evans syndrome (ES) was made. The patient was treated with intravenous immunoglobulin and high-dose methylprednisolone, followed by oral steroids, resulting in clinical and laboratory remission. Conclusion This case directs us to consider Evans syndrome as a differential diagnosis in infants presenting with pancytopenia. We advocate an international database of these patients to increase awareness of any patient with suspected ES.