Recurrent Infection in a Young Female Patient Recently Diagnosed With Primary Evans Syndrome Without Neutropenia

Abstract

Evans syndrome (ES) is a rare autoimmune condition of unknown etiology that occurs in a small subset of patients diagnosed, either sequentially or concomitantly, with immune thrombocytopenia (ITP) or warm autoimmune hemolytic anemia (AIHA). Neutropenia is present occasionally. Diagnosis is based on exclusion with a median age of 52 years of age. Here we have a case of a young patient with ES presenting with recurrent infection. ES should be included in differential diagnoses for patients presenting with AIHA, ITP, cytopenias or recurrent infection as the prognosis is more favorable when diagnosis is made early and symptoms are still mild.

Keywords: Autoimmune cytopenia; Autoimmune hemolytic anemia; Coombs test; Immune thrombocytopenia; Immunoglobulins; Neutropenia; Thrombocytopenia.

Figure 1

Increased densities along the middle to lower lung fields with patchy to reticular nodular density changes (red arrows).

Figure 2

Densities in the middle and lower lung fields (red arrows).