Our Summary

This research paper discusses a study conducted on children with sickle cell anemia, a blood disorder, who had their spleens removed (a procedure called splenectomy) between 2000 and 2018 at a hospital in Paris, France. The purpose of the study was to understand the risks and benefits of splenectomy in children, as well as the best age to perform this surgery.

The study included 188 children, with the average age of splenectomy being around 4 years old. These children were followed up for an average of about 6 years after the surgery. The main reasons for removing the spleen were due to acute splenic sequestration (a sudden, painful enlargement of the spleen) and hypersplenism (overactive spleen).

Before the surgery, all patients were given penicillin and most were vaccinated against pneumococcus (a type of bacteria that can cause serious infections) to prevent infections. The study found that the rates of severe bacterial infection and blood clots were very low, regardless of the age when the spleen was removed.

However, the study found that children who had their spleens removed before the age of 3 had a higher likelihood of developing cerebral vasculopathy (a condition that affects the blood vessels in the brain) compared to those who had the surgery later.

The study also found that a higher percentage of children who had their spleens removed were treated with hydroxycarbamide, a medication for sickle cell anemia, suggesting that children with spleen complications may have more severe forms of the disease.

The researchers suggest that if necessary, splenectomy should not be delayed in children, as long as they are given the recommended vaccines to prevent infections. They also suggest that spleen complications in children may indicate severe forms of sickle cell anemia.

FAQs

  1. What is the suggested age for a safe splenectomy in children with sickle cell anemia?
  2. What were the main indications for splenectomy in the study conducted at Robert Debré University Hospital?
  3. Does splenectomy increase the risk of invasive bacterial infection and thrombo-embolic events in children with sickle cell anemia?

Doctor’s Tip

One helpful tip a doctor might tell a patient about splenectomy is to ensure they receive proper vaccinations, particularly against pneumococcal infections, both before and after the procedure. It is also important to continue with antibiotic prophylaxis and close monitoring for any signs of infection or thrombo-embolic events post-surgery. Additionally, early intervention for spleen complications in children with sickle cell anemia may be necessary to prevent further complications.

Suitable For

Patients with sickle cell anemia who experience acute splenic sequestration or hypersplenism are typically recommended for splenectomy. In this study, children with sickle cell anemia who underwent splenectomy had a low incidence of invasive bacterial infections and thrombo-embolic events, regardless of their age at splenectomy. It was also found that children who were splenectomized at a younger age (<3 years) had a higher proportion of cerebral vasculopathy compared to those splenectomized at an older age (≥3 years). This suggests that splenectomy should not be delayed in children with sickle cell anemia who experience spleen complications, as it may be an indicator of a more severe phenotype. Additionally, it was observed that a greater proportion of splenectomized children were treated with hydroxycarbamide, indicating a more severe phenotype in these patients.

Timeline

  • Before splenectomy: A child with sickle cell anemia may experience acute splenic sequestration or hypersplenism, leading to the need for splenectomy. The child may receive penicillin prophylaxis, pneumococcal vaccination, and possibly pneumococcal conjugate vaccine shots prior to the surgery.

  • After splenectomy: The child will be closely monitored for post-splenectomy events, such as invasive bacterial infections and thrombo-embolic events. The overall incidence of these events is low, with no pneumococcal infections reported. Children who undergo splenectomy may have a more severe phenotype, as indicated by a higher proportion of them being treated with hydroxycarbamide compared to non-splenectomized children. It is important to not delay splenectomy if indicated, as spleen complications in childhood may indicate a more severe form of sickle cell anemia.

What to Ask Your Doctor

  1. What are the potential risks and benefits of undergoing a splenectomy?
  2. At what age is it safe for a child with sickle cell anemia to undergo a splenectomy?
  3. What are the indications for splenectomy in children with sickle cell anemia?
  4. What vaccinations and prophylactic measures should be taken before and after splenectomy?
  5. What is the recommended follow-up care after undergoing a splenectomy?
  6. How does splenectomy impact the overall management and treatment of sickle cell anemia?
  7. Are there any long-term complications or side effects associated with splenectomy?
  8. How does splenectomy affect the risk of infections in children with sickle cell anemia?
  9. How does splenectomy impact the risk of thrombo-embolic events in children with sickle cell anemia?
  10. What alternative treatment options are available for children with sickle cell anemia who may benefit from splenectomy?

Reference

Authors: Mechraoui A, Ithier G, Pages J, Haouari Z, Ali L, Bonnard A, Benkerrou M, Missud F, Koehl B, Holvoet L, Le Roux E, Brousse V. Journal: Haematologica. 2023 Dec 1;108(12):3409-3417. doi: 10.3324/haematol.2022.282556. PMID: 37226714