Does a Splenectomy Fix Red Blood Cell Shape in HE?

If you have hereditary elliptocytosis (HE), having your spleen removed (a procedure called a splenectomy) will not change the shape of your red blood cells. Because HE is caused by a genetic mutation that affects the proteins building the outer shell (membrane) of your cells, your red blood cells will always be oval or elliptical. However, for the small percentage of patients who experience severe symptoms, removing the spleen is a highly effective treatment. It stops the premature destruction of these oval-shaped cells, dramatically improving your anemia and extending the lifespan of your blood cells ^[1].

Why the Shape Doesn’t Change

Hereditary elliptocytosis is a genetic condition. It alters the DNA instructions for the structural proteins that give red blood cells their typical flexible, round shape. Even after a splenectomy, your body continues to follow these genetic instructions, producing red blood cells that are oval or elliptical instead of round ^[1]. Surgery cannot alter your genetics, so the physical shape of the cells remains the same.

How a Splenectomy Helps (If It Doesn’t Fix the Shape)

To understand why a splenectomy is recommended for severe cases of HE, it helps to understand what the spleen does. It is important to note that most people with HE have mild or no symptoms and will never need this surgery.

The spleen acts as your body’s primary blood filter. Normally, it removes old, damaged, or abnormally shaped red blood cells from circulation ^[1]. In HE, the spleen mistakenly sees your elliptical red blood cells as “defective” and destroys them prematurely. This rapid breakdown of red blood cells is called hemolysis.

When your red blood cells are destroyed faster than your body can make new ones, it leads to anemia (a low red blood cell count) ^[2]. By removing the spleen:

• You eliminate the main filter that is rapidly destroying your red blood cells ^[1].
• The liver partially takes over the job of filtering your blood, but it does not destroy the elliptical cells nearly as aggressively as the spleen did ^[3][4].
• The misshapen (but functional) elliptical cells are allowed to stay in your bloodstream much longer ^[2][5].
• Your overall red blood cell count rises, and symptoms of severe anemia—like extreme fatigue or shortness of breath—improve significantly ^[6][7].

While your severe symptoms will improve, you should expect that a mild anemia may persist because the underlying cell defect is still present and the liver continues to clear some of the cells ^[3][8]. In short, a splenectomy doesn’t cure the genetic shape of your cells or completely cure the anemia, but it successfully stops the dangerous, excessive destruction of your blood cells.

Important Considerations Before Surgery

While a splenectomy can greatly improve severe anemia and reduce the need for blood transfusions, it is a major procedure that introduces long-term health changes:

• Infection Risk: The spleen plays a vital role in your immune system. Without it, you will have a lifelong increased risk of severe infections, especially from encapsulated bacteria like pneumococcus, meningococcus, and Haemophilus influenzae ^[2][9].
• Vaccination Timeline: To protect your immune system, you will need specific vaccinations to target these bacteria. These vaccines must be given at least two to several weeks before the surgery to be fully effective ^[9]. You may also need to take preventative antibiotics afterward.
• Risk of Blood Clots (Thrombosis): Removing the spleen introduces a known, long-term risk of developing blood clots, such as deep vein thrombosis (DVT) or pulmonary embolism (PE) ^[10][11]. Patients with hereditary hemolytic anemias are especially vulnerable to this hypercoagulable state ^[12]. You will need to discuss ongoing monitoring and preventative measures with your hematologist.