Partial vs. Total Splenectomy in Hereditary Spherocytosis

Not every patient with hereditary spherocytosis (HS) needs surgery. However, for those with moderate to severe anemia who do, families and patients face a critical decision: should they undergo a total splenectomy (removing the entire spleen) or a partial splenectomy (removing only part of it)?

While partial splenectomies are primarily performed in young children to preserve their developing immune systems, the core trade-offs apply to anyone considering the procedure. Total splenectomy is considered the most definitive way to halt the anemia and reduce jaundice, though it does not change the underlying genetic shape of the red blood cells ^[1]. However, it permanently removes a vital immune organ. Partial splenectomy is a compromise: it leaves enough spleen tissue behind to help fight off infections, while removing enough to reduce the destruction of red blood cells ^[2][3]. Because a piece of the spleen is left behind, it can grow back over time, which may cause symptoms to return and require a second surgery ^[1][4].

The Threat of Infection (OPSI)

The spleen acts as a critical blood filter that helps the immune system recognize and destroy certain types of bacteria. Removing it entirely leaves a patient with a lifelong, elevated risk for Overwhelming Post-Splenectomy Infection (OPSI). OPSI is a rare but severe bacterial infection that can rapidly become a medical emergency. To mitigate this risk, doctors usually delay total splenectomies until a child is at least 5 or 6 years old to allow their immune system time to mature ^[5]. With proper preventative vaccines and antibiotics, the risk of OPSI is highly manageable, but any high fever (often defined as 101°F or 38.3°C, or as directed by your doctor) requires an immediate trip to the emergency room ^[5].

The primary benefit of a partial splenectomy is preserving the body’s immune function. By keeping a portion of the spleen intact, the risk of OPSI drops significantly ^[4][2]. Long-term studies of children who had a partial splenectomy have reported zero cases of OPSI during their follow-up periods ^[4][6]. However, because the remaining piece of the spleen can sometimes lose function over time, patients undergoing a partial splenectomy are still advised to receive the same specialized preventative vaccinations (like pneumococcal and meningococcal) just to be safe ^[5].

The Risk of Regrowth and Second Surgeries

While a partial splenectomy minimizes the danger of severe infections, its biggest drawback is the risk of symptom relapse. Spleen tissue is highly regenerative, meaning the small piece left behind can slowly grow back.

If the spleen grows large enough again, it will resume destroying red blood cells, causing anemia and fatigue to return ^[1]. Research shows that total splenectomy provides a more significant long-term improvement in red blood cell health and reduced bilirubin (the compound that causes jaundice) compared to partial splenectomy ^[1][7].

Because of this regrowth, patients who undergo partial splenectomy may eventually need a completion splenectomy (a second surgery to remove the remaining spleen tissue). Studies estimate that between 24% and 29% of pediatric patients who have a partial splenectomy eventually require this second operation ^[4]. When this regrowth happens, it typically occurs over a few years, with research showing the second surgery happening at a median of about three and a half to four years after the initial procedure ^[4].

The Gallstone Factor

Another critical consideration in this comparison is the ongoing risk of gallstones. In hereditary spherocytosis, the rapid breakdown of fragile red blood cells overloads the gallbladder with bilirubin, which forms gallstones. Doctors will typically perform an ultrasound before surgery to check for these stones. If they are already present, the gallbladder is often removed at the same time as the initial spleen surgery to save the patient from a future operation ^[4].

Because partial splenectomy does not always stop the destruction of red blood cells completely, patients are still at a higher risk of developing new gallstones in the future compared to those who have a total splenectomy ^[1]. If the gallbladder is not removed during the initial partial splenectomy, studies show there is up to a 39% chance the patient will need a delayed gallbladder removal surgery later on ^[4].

Weighing the Options

Ultimately, the choice involves trading one type of risk for another:

• Total Splenectomy: Offers the best chance of definitively halting the anemia and preventing future gallstones ^[1], but carries a lifelong risk of OPSI that requires constant vigilance, vaccines, and rapid medical attention for fevers ^[5].
• Partial Splenectomy: Maintains crucial immune defense against severe infections ^[2][6], but carries roughly a 1 in 4 chance of needing a second spleen surgery within a few years, alongside an ongoing risk of gallstones ^[4].