International Journal For Multidisciplinary Research

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

Author(s) Anubhav Deswal, Muskan Panwar
Country India
Abstract 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)
Keywords Evans Syndrome, AIHA, ITP, Autoimmune Neutropenia, Autoimmune Pancytopenia
Field Medical / Pharmacy
Published In Volume 6, Issue 5, September-October 2024
Published On 2024-10-25
Cite This Case Report of Evans Syndrome (AIHA and ITP with Immune Neutropenia: Autoimmune pancytopenia) - Anubhav Deswal, Muskan Panwar - IJFMR Volume 6, Issue 5, September-October 2024. DOI 10.36948/ijfmr.2024.v06i05.28056
DOI https://doi.org/10.36948/ijfmr.2024.v06i05.28056
Short DOI https://doi.org/g8pnrn
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