When do patients with sickle cell disease typically require splenectomy?

In patients with sickle cell disease, the spleen is particularly vulnerable to damage due to the repeated vaso-occlusive crises that result from the sickling of red blood cells. Over time, this leads to functional asplenia or autoinfarction of the spleen, meaning that the spleen becomes progressively non-functional as it undergoes repeated episodes of infarction.

This process often begins in early childhood, as the spleen becomes less effective in filtering blood and managing immune responses. By the time individuals reach early adulthood, many with sickle cell disease display features of auto-splenectomy, where the splenic tissue is essentially absent functionally due to chronic damage, although the physical organ may still be present.

Therefore, while splenectomy in the traditional sense (surgical removal) is not commonly required, most patients with sickle cell disease experience functional asplenia early in life, which has significant implications for their management, particularly regarding susceptibility to infections. Hence, recognizing that patients with this condition usually auto-splenectomize is crucial for understanding their clinical needs and guiding preventive care, such as vaccinations against encapsulated organisms (like Streptococcus pneumoniae).