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Evans Syndrome: Background, Clinical Presentation, Pathophysiology, and Management

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Immune Hematology

Abstract

Evans syndrome (ES) is a descriptive diagnostic term encompassing autoimmune disorders which affect two or more blood cell lines, resulting most commonly in autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) but also autoimmune neutropenia (AIN) in some cases. Since Evans and colleagues first described this phenomenon in the 1940s, classically defined ES has expanded to include the occurrence of multilineage autoimmune cytopenias either concurrently or sequentially. Furthermore, advances in the understanding of disease pathogenesis have uncovered many of the underlying etiologies responsible for the autoimmune cytopenias which define this syndrome, such that “idiopathic” is no longer a defining feature of the syndrome. Some of the most common pathologies now known to drive ES include autoimmune lymphoproliferative syndrome (ALPS), common variable immunodeficiency (CVID), and other systemic autoimmune diseases, all with the commonality of a dysregulated immune system in which autoreactive lymphocytes are poorly controlled, ultimately predisposing to autoimmunity. The recent advances in understanding of the pathologies driving ES have allowed for improved insight into optimal diagnostic and management strategies, although work is ongoing in the quest for continued improvement in understanding of disease pathogenesis and better outcomes for patients with ES.

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    Amanda B. Grimes MD

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    Jenny M. Despotovic

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Grimes, A.B. (2018). Evans Syndrome: Background, Clinical Presentation, Pathophysiology, and Management. In: Despotovic, J. (eds) Immune Hematology. Springer, Cham. https://doi.org/10.1007/978-3-319-73269-5_7

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