Case Report of Evans Syndrome (AIHA and ITP with Immune Neutropenia: Autoimmune pancytopenia)

Abstract Evans Syndrome is a very rare autoimmune disorder in which the immune system destroys the body's red blood cells, white blood cells, and/or platelets. The exact cause of this condition is unknown. It can be primary (no underlying cause identified) or secondary (associated with underlying disease). (1) Secondary causes can include SLE, MCTD, autoimmune lymphoproliferative syndrome, Sjogren, Non-Hodgkin lymphoma, CLL, antiphospholipid syndrome, HIV, and hepatitis C. Evans syndrome was first described in 1951 by R.S. Evans and is characterized by AIHA (autoimmune hemolytic anemia) and ITP (immune thrombocytopenic purpura) with or without neutropenia (leukopenia). In the U.S., this disease is estimated to affect fewer than 5000 individuals. Incidence increased from 0.97 (in 1980) to 1.84 (in 2016) per million years. Prevalence increased from 3.30 (in 1980) to 21.30 (in 2016) per million years. Common symptoms include autoimmune hemolytic anemia, autoimmune thrombocytopenia, autoimmune neutropenia, and petechiae. First-line treatment includes steroids and IVIG. Second-line treatment includes Rituximab. For refractory cases, treatments may include cyclosporine, MMF, cyclophosphamide, azathioprine, and HSCT. (2)