Our Summary

This research paper discusses a study done on children with autoimmune cytopenia (AIC), a condition where the body’s immune system attacks its own blood cells. The researchers looked at the results of a surgical procedure called a splenectomy (removal of the spleen) in these children. This procedure is effective in about 70-80% of children with a specific subtype of AIC known as chronic immune thrombocytopenia (cITP). However, there is less information about its effectiveness in children with other subtypes of AIC, such as autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES).

The decision to perform a splenectomy is difficult because it is irreversible and there is uncertainty about long-term outcomes. The researchers studied factors that may be associated with outcomes of splenectomy in these children.

The main outcome they measured was failure-free survival (FFS), which is the time from splenectomy to the start of a second-line treatment (other than steroids and intravenous immunoglobulins) or death. They included 161 patients in the study and followed them for a median of 6.8 years after splenectomy.

The subtype of AIC did not seem to affect FFS. However, they found that presence of immunopathological manifestations (IMs) - abnormal immune reactions - were strongly associated with worse outcomes. If a child was diagnosed with an IM before the splenectomy, they had a lower FFS. The risk was even higher if they were diagnosed with an IM at any time during follow-up. These children also had a higher risk of recurrent or severe bacterial infections and blood clots.

The researchers conclude that looking for associated IMs when considering a splenectomy could help weigh the risks and benefits. After the procedure, monitoring IMs could help identify patients at higher risk of worse outcomes.

FAQs

  1. What is the effectiveness of splenectomy in pediatric chronic immune thrombocytopenia (cITP) cases?
  2. What factors are associated with splenectomy outcomes in children with autoimmune cytopenia (AIC)?
  3. How are immunopathological manifestations (IMs) associated with the outcomes of a splenectomy?

Doctor’s Tip

A helpful tip a doctor might tell a patient about splenectomy is to be aware of the potential risk of immunopathological manifestations (IMs) after the procedure. Monitoring for IMs can help identify patients at higher risk of unfavorable outcomes, such as recurrent or severe bacterial infections and thrombosis. It is important to discuss these potential risks with your healthcare provider and to have regular follow-up appointments to monitor for any complications.

Suitable For

Patients with pediatric chronic immune thrombocytopenia (cITP), autoimmune hemolytic anemia (AIHA), and Evans syndrome (ES) are typically recommended splenectomy. Splenectomy is effective in approximately 70% to 80% of pediatric cITP cases, and there is limited data on its efficacy in AIHA and ES. The decision to undergo splenectomy in children with these conditions can be challenging due to the irreversible nature of the procedure and uncertainty about long-term outcomes.

Factors associated with splenectomy outcomes in pediatric patients with autoimmune cytopenia (AIC) include the presence of immunopathological manifestations (IMs). Patients diagnosed with an IM before or after splenectomy have a lower failure-free survival (FFS) rate, indicating a higher risk of needing second-line treatments or experiencing adverse events. Monitoring for IMs before and after splenectomy can help identify patients at higher risk for unfavorable outcomes.

In conclusion, patients with cITP, AIHA, and ES who have failed initial treatments may be recommended splenectomy, but close monitoring for IMs is crucial to assess the risk-benefit ratio and optimize outcomes.

Timeline

Before splenectomy:

  • Patient presents with symptoms of autoimmune cytopenia (such as low platelet count, anemia, or both)
  • Patient undergoes various treatments such as steroids, intravenous immunoglobulins, and other medications
  • Decision is made to proceed with splenectomy after failure of first-line treatments or due to severe symptoms

After splenectomy:

  • Patient undergoes splenectomy surgery to remove the spleen
  • Recovery period post-surgery, with monitoring for any complications
  • Monitoring for the development of immunopathological manifestations (IMs) which can indicate unfavorable outcomes
  • Regular follow-up appointments to assess the need for additional treatments or interventions
  • Long-term monitoring for any potential complications or risks associated with splenectomy, such as recurrent or severe bacterial infections and thrombosis.

What to Ask Your Doctor

  1. What is the success rate of splenectomy in treating my specific autoimmune cytopenia condition (e.g. chronic immune thrombocytopenia, autoimmune hemolytic anemia, Evans syndrome)?
  2. What are the potential risks and complications associated with splenectomy?
  3. How will my immune system be affected by the removal of my spleen?
  4. What alternative treatment options are available besides splenectomy?
  5. How long will it take to recover from splenectomy and what can I expect during the recovery process?
  6. Will I need to take any medications or undergo any additional treatments after the splenectomy?
  7. How often will I need to follow up with you after the procedure?
  8. Are there any specific lifestyle changes or precautions I should take after the splenectomy to reduce the risk of infections or other complications?
  9. What signs or symptoms should I watch for that may indicate a complication after the splenectomy?
  10. How will my long-term prognosis be affected by undergoing a splenectomy for my autoimmune cytopenia condition?

Reference

Authors: Pincez T, Aladjidi N, Héritier S, Garnier N, Fahd M, Abou Chahla W, Fernandes H, Dichamp C, Ducassou S, Pasquet M, Bayart S, Moshous D, Cheikh N, Paillard C, Plantaz D, Jeziorski E, Thomas C, Guitton C, Deparis M, Marie Cardine A, Stephan JL, Pellier I, Doré E, Benadiba J, Pluchart C, Briandet C, Barlogis V, Leverger G, Leblanc T. Journal: Blood. 2022 Jul 21;140(3):253-261. doi: 10.1182/blood.2022015508. PMID: 35443028