Asplenia syndrome

Asplenia syndrome (also known as right isomerism or Ivemark syndrome) is a type of heterotaxy syndrome.

There is an increased male predilection. Asplenia syndrome is usually diagnosed in neonates.4

In contrast to polysplenia syndrome, most patients die before 1 year-old because of severe/complexe congenital heart disease. Most patients are immunocompromised due to absent spleen.

Howell-Jolly and Heinz bodies are seen on H&E blood smear 6.

Main characteristic radiographic features include:

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Article information

rID: 25433
System: Paediatrics
Section: Gamuts, Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • Right isomerism
  • Asplenia syndrome
  • Right sided isomerism
  • Bilateral ride-sidedness

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