3 Things to Consider Prior to Removing Your Spleen for Spherocytosis

Liver, Gallbladder, Pancreas, Spleen
Liver, Gallbladder, Pancreas, Spleen. PIXOLOGICSTUDIO/SCIENCE PHOTO LIBRARY/Getty Images

Hereditary spherocytosis is a form of anemia that results from a change in the red blood cell membrane that prevents them from maintaining their normal shape. Red blood cells should look like a doughnut, but in hereditary spherocytosis they lose this shape and become spheres or ball-shaped. This change causes the red blood cell to be destroyed more rapidly, a process known as hemolysis.  

Because most of the hemolysis occurs in the spleen, splenectomy (surgical removal of the spleen) has been the mainstay of treatment for many years.

In fact, in years past, almost every patient with hereditary spherocytosis had their spleens removed. We now understand better some of the risks of splenectomy which must be weighed with the benefits. What should you consider before removing your spleen?

Risk of Infection

Your spleen is a major organ in your immune system. It assists in the process of making antibodies either due to infections or vaccinations. It also removes bacteria from the bloodstream, particularly types called encapsulated bacteria. When the spleen is removed, the body cannot remove these bacterial well which may result in severe, life-threatening infections.  

The two most common encapsulated bacteria are pneumococcus and meningococcus. To prevent these infections you should receive some additional immunizations prior to splenectomy. The pneumococcal vaccines are called Prevnar 13 and Pneumovax 23. There are two main types of meningococcal vaccines MCV4 (called Menveo or Menactra) and MenB (called Trumenba, or Bexsero).

To give the body time to make antibodies to these bacteria, It is best to receive these immunizations at least 2 weeks prior to splenectomy.

Despite these immunizations, after splenectomy you are at risk of overwhelming sepsis, a life-threatening bacterial infection. Although this is a rare event occurring in less than 2% of people after splenectomy, more than half will die despite intensive treatment.

The risk is highest in the first couple of years after splenectomy, but it can occur decades afterward. After splenectomy, your physician may prescribe twice daily penicillin to try to prevent these serious infections. Most importantly, after splenectomy fever is a medical emergency.  All fevers should be evaluated by a medical professional so that antibiotics may be given in case there is an infection.  

Knowing about the risk of infection is important and before your procedure, you should take time to discuss the risk with your doctor and decide if the procedure is for you.


Thrombosis, or blood clot, is a known complication of splenectomy. In particular, the portal vein, which drains blood from the GI system, and the splenic vein are at high risk of developing a thrombus. In general, the risk is highest in the first two weeks after splenectomy, but the risk remains lifelong.

The risk appears to be higher in patients who have their spleen removed for hereditary spherocytosis , other hemolytic anemias, or myeloproliferative disorders compared to those removed secondary to trauma.

The reason for this increased risk is not fully understood.  

Part of the risk is likely related to the development of thrombocytosis, an elevated platelet count, post-splenectomy. For the large majority of patients this will not cause any issues. However, some patients experience extreme thrombocytosis after splenectomy and may develop thrombosis secondary to platelet clumping.  

Pulmonary Hypertension

Most recently, there are concerns that splenectomy increases the risk of developing pulmonary hypertension, a condition of elevated blood pressure in the arteries of the lungs. It is not clear why pulmonary hypertension develops after splenectomy. It may occur secondary to the development of small clots in the blood vessels in the lungs. At this point, there are no recommendations for screening for pulmonary hypertension after splenectomy.  

Talk to Your Physician

A better understanding of these risks has led to a case-by-case approach to splenectomy. Significantly fewer patients are currently undergoing splenectomy. Despite these risks, splenectomy is still appropriate for some patients with hereditary spherocytosis.  

Patients who receive numerous blood transfusions, have recurrent pain, or difficulty gaining weight due to the size of the spleen may all benefit from spleen removal. Talk to your physician so that you understand these risks and benefits specific to your particular situation. 


Mentzer WC. Hereditary spherocytosis: Clinical features, diagnosis, and treatment. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on April 14, 2016).

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