Evans syndrome

Last revised by Frank Gaillard on 27 Feb 2024

Evans syndrome is an autoimmune condition with two or more cytopenias, usually caused by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia.

Evans syndrome is considered rare, with defining bicytopenias present in less than 5% of all patients diagnosed initially with either ITP or AIHA. The mean age at the time of diagnosis is 52 years, and it is slightly more common in females.

The clinical features of Evans syndrome are those of the bicytopenias and other hematopoietic cell lines affected. For example:

  • anemia: pallor, fatigue, jaundice, etc.

  • thrombocytopenia: easy bruising and bleeding, rarely more serious hemorrhage

  • neutropenia: increased risk and frequency of infections

Evans syndrome is generally sporadic, however, it can very rarely be inherited; familial Evans syndrome.

When sporadic, it is usually considered an idiopathic autoimmune condition against hematopoietic cells. When autoimmune hemolytic anemia is present, this is due to IgG antibodies reacting with red blood cell surface antigens at body temperature, and when immune thrombocytopenia is present, there are antibodies directed against GPIIb/IIIa on platelets.

Evans syndrome is often divided into either primary (idiopathic) or secondary forms. Secondary causes include:

Management options include:

  • supportive care, e.g. blood transfusions

  • immunosuppression, e.g. corticosteroids, intravenous immunoglobulin, rituximab

Additionally, if Evans syndrome is a secondary phenomenon, management should also be directed towards the underlying cause.

Evans syndrome was first described in 1951 by the American hematologist Robert "Bud" Evans (1912-1974) and associates 2,3.

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.