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Evans Syndrome Differential Diagnoses

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

Diagnostic Considerations

Evans syndrome may not be apparent when a child presents with an isolated cytopenia. Therefore, diagnosis may be delayed until a second episode of cytopenia manifests.

Evans syndrome is a diagnosis of exclusion. Other causes of immune cytopenias must therefore be ruled out before the diagnosis can be made. In addition to the conditions listed in the differential diagnosis, other problems to be considered include the following:

  • Immunoglobulin A (IgA) deficiency
  • AIDS
  • Autoimmune lymphoproliferative syndrome (ALPS)
  • Paroxysmal nocturnal hemoglobinuria
  • Thrombotic thrombocytopenic purpura
  • Hypersplenism
  • Rosai-Dorfman disease (may be associated with ALPS)

Differential Diagnoses

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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