Introduction:
Evans syndrome (ES), a hematological entity described by Evans and
colleagues in 1951, is characterized by the presence of Coombs positive
hemolytic anemia and immune thrombocytopenia, and less commonly,
autoimmune neutropenia [1]. Although the exact pathophysiology of ES
remains unknown, immune dysfunction with subsequent production of
antibodies targeting the erythrocytes and platelets is a likely
mechanism. Glucocorticoids and intravenous immunoglobulin (IVIG) have
mostly been used as first-line therapy for ES. Other treatment options
include immunosuppressants, blood transfusion, splenectomy, and
hematopoietic stem cell transplant [2]. ES has a more variable
clinical course as compared to isolated autoimmune hemolytic anemia
(AIHA), with more frequent exacerbations and mortality [3]. The
co-existence of tuberculosis (TB) with Evans syndrome makes therapy even
more challenging.