Our Summary

Hereditary spherocytosis (HS) is a common inherited disorder that results in anemia, caused by a malfunction in the red blood cell membrane. This study looked at different types of surgeries used to treat HS, and found that there was no significant difference in patient outcomes between the different types of surgeries, based on measurements of things like red and white blood cell counts and levels of certain substances in the blood. However, they did notice that patients had high levels of platelets, which help the blood to clot, for the first 6 months after surgery, which suggests that these patients may benefit from taking oral anti-platelet therapy during this time. The study concludes that a type of surgery called laparoscopic splenectomy could be a good treatment choice for HS.

FAQs

  1. What is Hereditary spherocytosis (HS) and how is it caused?
  2. What is the impact of different types of surgeries on patient outcomes in the treatment of HS?
  3. What is the significance of high levels of platelets in patients after surgery and how can it be managed?

Doctor’s Tip

One helpful tip a doctor might tell a patient about splenectomy is to be vigilant about preventing infections, as the spleen plays a key role in the body’s immune system. Patients who have had their spleen removed are at a higher risk for infections, particularly from certain bacteria such as Streptococcus pneumoniae. It is important for these patients to stay up to date on vaccinations, especially the pneumococcal vaccine, and to practice good hygiene to reduce the risk of infections. Additionally, patients should seek medical attention promptly if they develop signs of infection, such as fever, chills, or unusual fatigue.

Suitable For

Patients with hereditary spherocytosis (HS) are typically recommended splenectomy as a treatment option. Other conditions that may warrant splenectomy include:

  1. Idiopathic thrombocytopenic purpura (ITP): a condition characterized by low platelet counts and an increased risk of bleeding.

  2. Autoimmune hemolytic anemia: a condition where the immune system attacks and destroys red blood cells.

  3. Sickle cell disease: a genetic disorder that causes red blood cells to become misshapen and break down easily.

  4. Thalassemia: a genetic disorder that affects the production of hemoglobin, leading to anemia.

  5. Hodgkin’s lymphoma: a type of cancer that affects the lymphatic system.

  6. Non-Hodgkin’s lymphoma: another type of cancer that affects the lymphatic system.

  7. Chronic lymphocytic leukemia: a type of cancer that affects white blood cells.

  8. Gaucher disease: a genetic disorder that causes the accumulation of fatty substances in cells and organs.

  9. Hereditary elliptocytosis: a rare genetic disorder that affects the shape of red blood cells.

It is important for patients to discuss their individual case with their healthcare provider to determine if splenectomy is the best treatment option for their condition.

Timeline

Before splenectomy:

  1. Patient may experience symptoms of anemia, such as fatigue, weakness, and pale skin.
  2. Patient may undergo blood tests to diagnose the underlying cause of their anemia, which may lead to a diagnosis of hereditary spherocytosis.
  3. Patient may receive treatment for anemia, such as blood transfusions or medication, to manage their symptoms before surgery.

After splenectomy:

  1. Patient undergoes laparoscopic splenectomy surgery to remove the spleen.
  2. In the first 6 months after surgery, patient may experience high levels of platelets in the blood, which can increase the risk of blood clots.
  3. Patient may benefit from taking oral anti-platelet therapy during this time to reduce the risk of blood clots.
  4. Patient may have regular follow-up appointments with their healthcare provider to monitor their blood counts and overall health post-surgery.

What to Ask Your Doctor

  1. What are the potential risks and complications associated with a splenectomy for treating hereditary spherocytosis?

  2. How long is the recovery process after a splenectomy surgery?

  3. Will I need to take any medications or make any lifestyle changes after the surgery?

  4. How will a splenectomy impact my immune system and my risk of infections?

  5. Are there any long-term effects or complications I should be aware of after having a splenectomy?

  6. How often will I need to follow up with you after the surgery?

  7. Are there any alternative treatments or therapies for hereditary spherocytosis that I should consider before opting for a splenectomy?

  8. Will I need any vaccinations or special precautions after having my spleen removed?

  9. Will I need to make any dietary changes or adjustments to my physical activity after the surgery?

  10. What is the success rate of a laparoscopic splenectomy compared to other types of surgeries for treating hereditary spherocytosis?

Reference

Authors: Zaharie F, Muresan MS, Tomuleasa C, Popa G. Journal: Ann Ital Chir. 2018;89:569-571. PMID: 30665220