Evans Syndrome Clinical Presentation

Updated: Feb 03, 2020
  • Author: Ragini Malika Adams, MD; Chief Editor: Hassan M Yaish, MD  more...
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History and Physical Examination

In a national survey, thrombocytopenia was the presenting feature in 76% of patients with Evans syndrome, and anemia was the presenting feature in 67% of these patients. [28] In addition, 24% of patients had neutropenia, and 14% had pancytopenia.

Evans syndrome is a diagnosis of exclusion. Confounding disorders, such as infections, rheumatologic diseases, and malignancies, can present with autoimmune cytopenias and must be ruled out.

Signs of thrombocytopenia include purpura, petechiae, and ecchymoses. Signs of anemia include pallor, fatigue, and light-headedness. Jaundice may indicate hemolysis.



Potential complications of Evans syndrome include the following:

  • Hemorrhage with severe thrombocytopenia – The national survey reported hemorrhage in 29% of patients, [28] with 2 deaths resulting from severe GI bleeding and a third death from acute intracranial bleeding

  • Serious infection in patients with neutropenia – The national survey showed invasive infections in 29% of patients, including pneumonia, sepsis, meningitis with Streptococcus pneumoniae, localized abscess, and osteomyelitis [28] ; one patient died of presumed sepsis and liver failure 9 years after splenectomy