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Evans syndrome

Orpha number ORPHA1959
Synonym(s) -
Prevalence 1-9 / 1 000 000
Inheritance
  • Sporadic
Age of onset Variable
ICD-10
  • D69.3
OMIM -
UMLS -
MeSH -
MedDRA
  • 10053873
SNOMED CT
  • 75331009

Summary

Evans syndrome is characterised by the association of autoimmune haemolytic anaemia with another haematological anomaly (such as destruction of the polynuclear neutrophils, neutropaenia or, more frequently, platelet destruction or thrombocytopaenia). The prevalence is estimated at 1 in 1,000,000. The thrombocytopaenia may precede, occur concurrently with, or secondary to the autoimmune haemolytic anaemia. Evans syndrome is a rare disease for which the treatment strategy is poorly defined. Management usually involves administration of immunosuppressors or splenectomy.

Expert reviewer(s)

  • Pr Guy LEVERGER

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Detailed information

Clinical practice guidelines
  • FR (2009,pdf)
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