Spleen is an organ in the abdomen that plays an important role in immune system being a key-role organ in fighting any germs in the blood, filters the blood, stores blood cells, and destroys old blood cells.
Splenectomy is the surgical removal of spleen and is a recommended intervention in patients with hemoglobinopathies patients when indicated. In case of patients with sickle cell disease (SCD) there are several reasons indicating splenectomy:
- acute splenic sequestration crisis (blood trapped in the spleen, usually seen in children)
- hypersplenism (a case of an overactive spleen, removing blood cells too early and too quickly)
- massive splenic infarction (a segment or even the whole spleen is necrotized) and
- splenic abscess (a walled-off infection within the spleen).
The decision to remove or not the spleen in patients with SCD is a very complex issue in choosing the right time and the right patient.
A study in 534 Italian patients with SCD followed up for 26 years showed that there were no difference in survival of patients undergone splenectomy versus those who did not have their spleen removed. Moreover, the rate of fatal infections among those splenectomized and non-splenectomized did not differ. Finally, there was no observed difference between the thrombotic complications in both groups.
The genotype of SCD [Hemoglobin S-disease (SS), HemoglobinS/β(+) thalassaemia (Sβ), Hemoglobin S/β(-) thalassemia (Sβ-)] is a crucial factor regarding the decision to remove the spleen. Patients with HemoglobinS/β(+) thalassaemia seem to require splenectomy more frequently than those with SS, whereas SS patients seem to undergo autosplenectomy.
This study shows that splenectomy does not have any negative impact in the outcome of patients with SCD and should be considered as a therapeutic option.
Finally, we have to underline that immunization status and antibiotic prophylaxis – both in splenectomized and non-splenectomized – and the follow-up in expertized centers is of a great significance.