Prognosis

Surprisingly little information is available about the long-term outcome of people with hereditary spherocytosis (HS) who have been followed for several decades, either with or without splenectomy. This may be because most patients do well and live a normal life, and therefore do not want or see the need for follow-up.

Deaths from overwhelming post-splenectomy sepsis are very rare.

Genetic counselling

Adults with HS, or parents of a child with HS who are contemplating further children, need to be advised about the risk of a child inheriting the disorder. HS is inherited dominantly in 75% of cases, and each child has a 1 in 2 or 50% risk of inheriting the abnormality.[2] In those with no previous family history of the condition, it will be more difficult to predict the outcome for future children. Demonstration of the mutation or mutations in the affected individual may be helpful, although this is currently only a research investigation.[19]​​

HS outcome after splenectomy

Most people are asymptomatic after splenectomy, with a normal or near-normal haemoglobin. Spherocytes are still seen in the blood smear, but the red-cell life span is much improved (often to normal).[46] Symptoms and signs of haemolysis are absent in all but the most severe cases.

There are long-term risks of infection and thrombosis after splenectomy. The infection risk is much reduced by appropriate pre-splenectomy vaccination, antibiotic prophylaxis, and prompt medical attention during episodes of fever.[7][46]​ Further study is required to fully assess the risk of vascular complications, but it appears from limited evidence that this risk is greatest many years after the splenectomy.[45][60]

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