Surgical hope for inherited anaemia

Surgery: A radical but sometimes necessary solution

Removing most of the spleen could help children suffering from inherited blood disorders, say researchers in the US.

They carried out surgery on 25 children born with a type of anaemia caused by abnormal red blood cells.

The condition - hereditary spherocytosis - leads to fatigue, jaundice, and repeated infections.

Until now there have been no large and conclusive studies showing the procedure can be performed safely with good outcomes

Dr Henry Rice, Duke University

If the disease is very severe, the patient's spleen may have to be removed.

Doctors at Duke University Medical Center and the Medical College of Wisconsin carried out a procedure that is seldom used in the US.

They found that by leaving 10-20% of the spleen intact, the organ continued to function, producing immune cells that protect the body from disease.

"The goal is to take away enough of the spleen to help relieve the anaemia and its symptoms, while leaving enough to maintain a viable immune function," said paediatric surgeon Dr Henry Rice.

"Until now there have been no large and conclusive studies showing the procedure can be performed safely with good outcomes."

Technical hurdle

Dr Rice said that while partial splenectomies have been carried out in Europe for the past decade, they have been slow to catch on in the US.

The procedure is technically difficult and some surgeons are concerned that the spleen may grow back again in time.

But Dr Rice said that none of the 25 children given the operation had suffered serious infections or needed further surgery. Some had been monitored for as long as six years, he said.

The research is published in the Annals of Surgery.