Evans Syndrome Differential Diagnoses

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

  • Common variable immune deficiency (CVID)

  • Paroxysmal nocturnal hemoglobinuria

  • Thrombotic thrombocytopenic purpura

  • Hypersplenism

  • Rosai-Dorfman disease (may be associated with ALPS)

Differential Diagnoses