Asplenia syndrome

Last revised by Rohit Sharma on 15 Aug 2023

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/complex 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:

It carries poor prognosis than left isomerism (polysplenia syndrome) as it has more association with congenital heart diseases. 1-year mortality is approximately 85% with right isomerism and more than 50% with polysplenia syndrome 7.

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