The spleen is an organ situated on the left side of the upper tummy, just under the ribcage. It is a small organ, which looks a bit like a kidney.
The function of the spleen
The spleen has three important functions:
- Fight infections
- Filter the blood
- Storage for blood
The blood flows through small veins into the spleen. The spleen contains two kinds of tissue: the white pulpa and the red pulpa. In the white pulpa (where there are a lot of white bloodcells), the blood will be screened for diseases and foreign cells, which will then be destroyed by the white blood cells.
Eventually, the blood will enter an ingenious system within the red pulpa, where the blood is pushed throught little holes with diameters of only 3 micrometers (µm). Because the red bloodcells are 4 µm, only the most flexible and healthy cells will go through the holes. Even with the smallest abnormality, the red blood cells won’t be able to twist themselves through the spleen and will be destroyed. The iron that is left when the red blood cells are broken down, is then stored within the spleen until it will be taken away for further use. Just like the spleen will also storage white blood cells and platelets.
What happens in the spleen when having Pyruvate Kinase Deficiency?
Patients with Pyruvate Kinase Deficiency have a higher amount of damaged red blood cells because their red blood cells are not stable and have a different shape and size. For that reason, when the blood is pushed through the little holes within the spleen, they will not fit and the red blood cell will be destroyed. We call this process haemolysis.
To compensate this higher workload, the spleen will expand. An enlarged spleen (also called splenomegaly) is very typical for patients with hemolytic anemia. Mostly, the swelling is not very severe, but in some cases the patient can have an enormous spleen. This can cause a vague feeling of oppression in the left side of the belly. Sometimes, it can also cause abdominal pain.
Spleen removal as a treatment for Pyruvate Kinase Deficiency
When a child is severely transfusion dependent, a possible treatment consists in removing the spleen (also called a splenectomy). It has been proven that people can function very well without the spleen, despite the functions it has. Since the spleen plays an important role in the destruction of the red blood cells, it’s removal often leads to a major improvement of the disease.
However it will not work for everybody. In some cases, a splenectomy will reduce the anemia and the patient won’t need blood transfusions anymore, in other cases there’ll only be an improvement in the lifespan of the red blood cells. It depends greatly on where in the body the haemolysis occurs most. In patients with a mild hemolytic anemia, the red blood cells are mostly eliminated by the spleen and a splenectomy will have good results. In patients with severe haemolysis, the red blood cells are also destroyed in other organs, particulary in the liver. Removing the spleen in those patients will be less advantage.
In some hospitals the doctors will do a clinical test first, where they give the patient radioactive red blood cells to show on a scan where the red blood cells are mostly destroyed.
“I had my spleen removed many years ago at the age of 2. Before that, I required transfusions every 3 weeks. It was life changing for me and my family. Even though splenectomy did not resolve all issues for me, I went about 42 years without regular transfusions. Now that I’m older, I do get transfusions occasionally.”
Female, 44 years
The risks of a spleen removal
A splenectomy is an chirurgical operation, where the doctors will take out the spleen completely. Before doctors will perform this surgery, they must be sure that the child has enough protection to fight infectious diseases. For that reason they will try to postpone the surgery until the child is older than 2 years old and has had al the important vaccines. If the child doesn’t have all the needed vaccines (for example when parents are against vaccination), the operation won’t be performed because of the high risks for infections.
One of the major risks of a splenectomy is the so called “overwhelming post-splenectomy infection (OPSI)”. As said before, the spleen has an important role to fight infections: it screens the blood for bacteria and foreign cells, which are then destroyed by the white blood cells in the spleen. When the spleen is no longer present, the bacteria won’t be removed from the blood circulation and will be free to cause infection. For that reason it is extremely important that patients without a spleen have regular vaccinations and take some safety precautions.
Vaccine recommendations for people without a spleen
The following vaccines are recommended for asplenic (without a spleen) individuals per the U.S. Centers for Disease Control and Prevention.
- Annual influenza vaccine
- Pneumococcal Conjugate vaccine – PCV13 (1 dose)
- Pneumococcal Polysaccharide vaccine – PPSV23 (1 dose, plus a booster dose 5 years later, and another dose after age 65)
- Meningococcal Conjugate vaccine – MCV4 (1 dose, plus boosters every 5 years)
- Haemophilus influenza B vaccine – Hib (1 dose)
Disclaimer: This article is written by patients and is meant for basic informational purposes only. It is not intended to serve as medical advice, substitute for a doctor's appointment or to be used for diagnosing or treating a disease. Users of this website are advised to consult with their physician before making any decisions concerning their health. For details see our full disclaimer.
Pyruvate Kinase Deficiency 