Our Summary

This research paper discusses a study on the effectiveness and complications of splenectomy, which is the removal of the spleen, in children with transfusion-dependent thalassemia (TDT), a blood disorder that requires regular transfusions. The study looked at cases from a 30-year period and followed up with the patients for several years after their surgery.

The study included 39 children, and the average age at the time of their surgery was about 11 years old. The researchers measured the effectiveness of the surgery by looking at the number of transfusions the patients needed in the first year after surgery and at their last follow-up appointment.

They found that none of the patients had a complete response, meaning the need for transfusions didn’t completely go away. About a third of the patients had a partial response, meaning they needed fewer transfusions, but the majority didn’t respond at all and still needed the same amount of transfusions.

Most of the patients experienced a condition called thrombocytosis, which means they had too many platelets in their blood. Some also experienced complications like blood clots, infections, and high blood pressure in the lungs.

The researchers concluded that splenectomy only reduced the need for transfusions in a small number of patients with TDT. They suggest that it might be better to delay the surgery and instead treat the patients with a type of therapy called chelation therapy, which can help manage the amount of transfusions they need.

FAQs

  1. What is the purpose of a splenectomy in patients with transfusion-dependent thalassemia (TDT)?
  2. What were the common complications observed in children with TDT after splenectomy?
  3. How effective is splenectomy in reducing the need for transfusion in TDT patients?

Doctor’s Tip

One helpful tip a doctor might tell a patient about splenectomy is to ensure they are up to date on vaccinations, particularly those recommended for individuals without a spleen. This includes vaccinations for pneumococcal, meningococcal, and Haemophilus influenzae type b infections to help prevent serious complications. It is also important for patients to seek medical attention promptly if they develop symptoms of infection, as they may be at a higher risk for certain types of infections after splenectomy.

Suitable For

Patients with transfusion-dependent thalassemia (TDT) who may be recommended for splenectomy include those who have not had a significant decrease in transfusion requirement with other treatment options, such as iron chelation therapy. Additionally, patients who experience complications related to splenomegaly, such as hypersplenism or portal hypertension, may also be considered for splenectomy. It is important to assess each patient individually and weigh the potential benefits and risks of splenectomy in order to make an informed decision.

Timeline

Before splenectomy:

  • Patient with transfusion-dependent thalassemia (TDT) may undergo a series of blood transfusions to manage their condition.
  • They may experience symptoms such as fatigue, weakness, and shortness of breath due to their anemia.
  • The decision to undergo splenectomy is made based on the patient’s medical history, transfusion requirements, and overall health status.

After splenectomy:

  • The patient undergoes the surgical procedure to remove their spleen.
  • In the first year post-splenectomy, the patient’s transfusion requirements are monitored to assess the effectiveness of the procedure.
  • Long-term follow-up involves monitoring for complications such as thrombocytosis, thrombosis, infections, and pulmonary hypertension.
  • The patient may still require blood transfusions after splenectomy, but the frequency and amount may decrease in some cases.
  • Aspirin prophylaxis may be given to manage thrombocytosis.
  • Overall, splenectomy may partially decrease the need for transfusions in a small number of TDT patients, but complete response is rare.

What to Ask Your Doctor

  1. What are the potential benefits of splenectomy for my condition?
  2. What are the risks and potential complications associated with splenectomy?
  3. How will splenectomy impact my transfusion requirements in the long term?
  4. Will I need to take any medications or undergo additional treatments after splenectomy?
  5. How will splenectomy affect my risk of developing infections or other complications?
  6. What is the long-term outlook for patients who undergo splenectomy for TDT?
  7. Are there any alternative treatment options that I should consider before proceeding with splenectomy?
  8. How often will I need follow-up appointments after splenectomy, and what will be monitored during these appointments?
  9. Are there any lifestyle changes or precautions that I should take after splenectomy?
  10. What is the experience of your team with performing splenectomy for TDT, and what is the success rate of the procedure in your practice?

Reference

Authors: Akca T, Ozdemir GN, Aycicek A, Ozkaya G. Journal: J Pediatr Hematol Oncol. 2023 Apr 1;45(3):143-148. doi: 10.1097/MPH.0000000000002468. Epub 2022 Apr 18. PMID: 35446800