Our Summary

This research paper discusses inherited blood disorders, specifically those causing hemolytic anemias, where the body destroys red blood cells faster than it can produce them. One treatment option is splenectomy, or removing the spleen, as it is responsible for removing damaged red blood cells.

The effectiveness of splenectomy in treating hereditary spherocytosis, one type of hemolytic anemia, is well-established. However, it’s unclear whether the same procedure is as effective for other types of hemolytic anemias. There are also concerns about possible complications from the surgery, including infection and blood clots.

The researchers provide specific recommendations for treating each disorder (except thalassemia syndromes, which have separate guidelines) to help doctors make better-informed decisions about the use of splenectomy. They also suggest what type of splenectomy should be used and discuss possible consequences of the surgery.

These recommendations are based on expert opinions, as there were no previous clinical trials or studies found on the use of splenectomy for these disorders. The recommendations were then carefully reviewed and adjusted by a group of hematologists who treat both adults and children with these rare anemias.

FAQs

  1. What is the role of the spleen in the development of hemolytic anemias?
  2. How effective is splenectomy in treating different types of hemolytic anemias?
  3. What are the possible complications of splenectomy surgery?

Doctor’s Tip

One helpful tip a doctor might tell a patient about splenectomy is to ensure they receive proper vaccinations before and after the surgery, as the spleen plays a key role in the body’s immune response. Patients should also be vigilant about monitoring for signs of infection post-surgery and seek immediate medical attention if any symptoms arise. Additionally, patients should follow up with their healthcare provider regularly to monitor their blood cell counts and overall health after undergoing a splenectomy.

Suitable For

Patients with hereditary spherocytosis, autoimmune hemolytic anemia, and other hemolytic anemias that are not responding well to other treatments may be recommended for splenectomy. This procedure may help alleviate symptoms and improve quality of life for these patients. However, it is important for patients to discuss the risks and benefits of splenectomy with their healthcare providers before making a decision.

Timeline

Timeline of patient experiences before and after splenectomy:

Before splenectomy:

  • Patient is diagnosed with a hereditary hemolytic anemia such as hereditary spherocytosis.
  • Patient experiences symptoms of anemia, such as fatigue, pale skin, and shortness of breath.
  • Patient undergoes various tests to confirm the diagnosis and determine if splenectomy is a suitable treatment option.
  • Patient and their healthcare team discuss the risks and benefits of splenectomy and decide to proceed with the surgery.

After splenectomy:

  • Patient undergoes the splenectomy surgery, which can be done laparoscopically or through open surgery.
  • Patient may experience pain and discomfort post-surgery, which is managed with pain medication.
  • Patient is monitored closely for any signs of infection or blood clots, which are potential complications of the surgery.
  • Patient may need to take antibiotics or blood thinners as part of their post-operative care.
  • Over time, the patient’s symptoms of anemia improve as the body no longer destroys red blood cells at an accelerated rate.
  • Patient may need to receive vaccinations to prevent infections, as the spleen is an important part of the immune system.
  • Patient continues to have regular follow-up appointments with their healthcare team to monitor their progress and overall health.

What to Ask Your Doctor

Some questions a patient should ask their doctor about splenectomy include:

  1. What type of hemolytic anemia do I have, and is splenectomy a recommended treatment option for my specific condition?
  2. What are the potential risks and complications associated with splenectomy, and how likely are they to occur in my case?
  3. How will removing my spleen affect my immune system and overall health in the long term?
  4. Are there alternative treatments or therapies available for my condition that do not involve splenectomy?
  5. What is the success rate of splenectomy in treating hemolytic anemias, and what outcomes can I expect after the surgery?
  6. How long will it take to recover from splenectomy, and what follow-up care will be required?
  7. Are there any specific lifestyle changes or precautions I should take after undergoing splenectomy?
  8. Will I need to receive any vaccinations or medications to prevent infections after splenectomy?
  9. How often will I need to follow up with my doctor after the surgery, and what signs or symptoms should I watch out for that may indicate complications?
  10. Can you provide me with more information or resources about splenectomy and its implications for my condition?

Reference

Authors: Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L, Pignata C, Graziadei G, Pospisilova D, Rees DC, de Montalembert M, Rivella S, Gambale A, Russo R, Ribeiro L, Vives-Corrons J, Martinez PA, Kattamis A, Gulbis B, Cappellini MD, Roberts I, Tamary H; Working Study Group on Red Cells and Iron of the EHA. Journal: Haematologica. 2017 Aug;102(8):1304-1313. doi: 10.3324/haematol.2016.161166. Epub 2017 May 26. PMID: 28550188