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Evans Syndrome Clinical Presentation

  • Author: Prasad Mathew, MBBS, DCH, FAAP; Chief Editor: Hassan M Yaish, MD  more...
Updated: Apr 11, 2016

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.[15] 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, 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,[15] 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[15] ; one patient died of presumed sepsis and liver failure 9 years after splenectomy
Contributor Information and Disclosures

Prasad Mathew, MBBS, DCH, FAAP Professor of Pediatrics, Division of Hematology/Oncology, University of New Mexico School of Medicine

Prasad Mathew, MBBS, DCH, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society on Thrombosis and Haemostasis, American Society of Clinical Oncology, National Hemophilia Foundation, Hemophilia and Thrombosis Research Society, International Society of Paediatric Oncology, World Federation of Hemophilia

Disclosure: Received salary from Bayer HC for payment for services rendered.

Chief Editor

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, New York Academy of Sciences, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society

Disclosure: Nothing to disclose.

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