Evans syndromeD69.31

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 11.11.2022

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Synonym(s)

Autoimmune hemolytic anemia thrombocytopenic purpura syndrome idiopathic; Evans-Fisher syndrome; Evans-Fisher Syndrome; idiopathic autoimmune hemolytic anemia and thrombocytopenic purpura syndrome; Primary thrombocytopenic purpura and acquired hemolytic anemia

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HistoryThis section has been translated automatically.

Evans, 1949

DefinitionThis section has been translated automatically.

Evans syndrome is an autoimmune disorder associated with two or more cytopenias, which usually include autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia (about 15% of cases).

AIHA, which occurs in Evans syndrome, is a heat IHA in which IgG antibodies react with red blood cell (RBC) surface antigens at body temperature, in contrast to cold AIHA. In ITP, the immune system targets GPIIb/IIIa on platelets.

ClassificationThis section has been translated automatically.

It has been suggested that the disease is:

  • primary (idiopathic)
  • or
  • secondary

sympotmatic, associated with an underlying disease.

Secondary Evans syndrome has been associated with diseases such as systemic lupus erythematosus (SLE), immunodeficiency syndromes (CVID), and autoimmune lymphoproliferative syndrome (ALPS) in non-Hodgkin's lymphoma (NHL) in patients over 50 years of age, chronic lymphocytic leukemia (CLL), viral infections (such as HIV, hepatitis C), and after allogeneic hematopoietic cell transplantation.

It is important to identify Evans syndrome as a secondary syndrome when it is associated with another disease, as cytopenias are more severe in Evans syndrome than in AIHA or ITP. Treatment options also differ depending on the classification.

EtiopathogenesisThis section has been translated automatically.

Pathogenetically, antibodies against erythrocytes and against platelets lead to accelerated peripheral degradation of these cells. The antibodies are not cross-reactive, i.e. they are not directed against a common antigen of erythrocytes and platelets.

Clinical featuresThis section has been translated automatically.

Anemia, icterus, possibly purpura.

LaboratoryThis section has been translated automatically.

Erythrocyte autoantibodies, platelet autoantibodies.

Differential diagnosisThis section has been translated automatically.

Purpura, thrombocytopenic.

LiteratureThis section has been translated automatically.

  1. Evans RS, Duane RT (1949) Acquired hemolytic anemia: relation of erythrocyte antibody to activity of the disease; significance of thrombocytopenia and leukopenia. Blood 4: 1196-1213
  2. Leitner GC et al (2003) Idiopathic autoimmune thrombocytopenia: evidence for redistribution of platelet antibodies into the circulation after immunoadsorption treatment. Am J Hematol 73: 44-47.
  3. Petrides PE, Hiller E (1992) Autoimmune hemolytic anemia combined with idiopathic thrombocytopenia (Evans syndrome). Clinical Investigator 70: 38-39

  4. Shaikh H et al (2022) Evans Syndrome. 2022 Jun 21. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing PMID: 30085557.

Authors

Last updated on: 11.11.2022

Author: Prof. Dr. med. Peter Altmeyer