Evans Syndrome

Excerpt

Evans syndrome is an autoimmune condition that presents with two or more cytopenias, which commonly includes autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia (only in 15% of cases, according to a report) . The type of AIHA that presents in Evans syndrome is warm AIHA, in which IgG antibodies react with red blood cell (RBC) surface antigens at body temperature, as opposed to cold AIHA. In ITP, the immune system is directed against GPIIb/IIIa on the platelets.

Recently, a proposition has been laid out to classify the condition as primary (idiopathic) or secondary (associated with an underlying disorder) . Secondary Evans syndrome has been associated with diseases such as systemic lupus erythematosus (SLE), common variable immunodeficiency (CVID), and autoimmune lymphoproliferative syndrome (ALPS) in Non-Hodgkin lymphoma (NHL) in patients older than 50 years, chronic lymphocytic leukemia (CLL), viral infections (such as HIV, hepatitis C) and following allogeneic hematopoietic cell transplantation. Identifying Evans syndrome as secondary when associated with a disease is important because cytopenias have been observed to be more severe when with Evans syndrome in contrast to when presenting alone as AIHA or ITP. Also, the treatment options differ according to the classification.