Search

In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study, newborns are screened for SCD and placed on oral penicillin prophylaxis in nearly all of the United States, as well as in other countries where the disease is highly prevalent. The previously described permanent, complete and nearly universal "autosplenectomy" emerging by late childhood or early adulthood is now challenged by recent findings of reversibility of splenic dysfunction by the antisickling drug hydroxyurea or by successful allogeneic stem cell transplantation, even in older patients. Imaging techniques for hypofunction of the spleen are the most commonly used modalities to guide the clinician in decisions regarding medical or surgical management.

Background: The spleen is one of the most frequently affected organ in Sickle cell anaemia (SCA). This has been attributed to its complex anatomy and prominent reticuloendothelial functions which include clearance of unwanted particulate matter in blood ( culling), defense against infection and reservoir for blood cells. This paper aims to highlight the current information on the changes that occur in the spleen of Sickle Cell Disease patients in this environment., Method: A review of relevant literature on the subject of splenic changes in Sickle Cell Disease sourced by manual library and medline search., Results: The essential splenic change in SCA is splenomegaly and subsequent shrinkage in size (autosplenectomy), which maybe due to several factors. These include: high levels of irreversible sickle cells, decreased HbF associated with increased intravascular sickling and chronic Malaria infection secondary to hyperplasia of the reticulo-endothelial system and increased antibody production especially IgG and IgM. Finally, the clinical complications of these splenic changes such as increased susceptibility to infection, acute splenic sequestration and hypersplenism are also reviewed in this paper., Conclusion: In view of the above changes, it is important to ensure regular monitoring and follow-up in order to prevent complications, recurrent crisis and death.

Although there is as yet no definitive treatment for patients with sickle cell disease there has been considerable improvement in the overall management of these patients in recent years. This stems largely from a better understanding of the disease process. The risks from infection in infancy and childhood due to asplenia ae now well known, and children at risk ar being identified and treated. The role of platelets and the activation of clotting factors, while still controversial, are now acknowledged as appropriate areas for research. The judicious use of blood transfusions for cerebral infarction has improved the prognosis for this complication, as it has for pregnancy and the fetus. Blood transfusion, however, has its own hazards in sickle cell disease and the extent to which it can be used requires further investigation. Finally, the broad spectrum of hematological findings and individual clinical variations in sickle cell disease is now better understood.

On the occasion of 34 spleen scintigrams performed in children with sickle cell anemia, the frequency and precocity of the "functional asplenia" is emphasized and its relationship with the infectious history of such children is discussed. The anti-bacterial role of the spleen and the responsibility, at least partial, or asplenia in the susceptibility of patients with sickle cell anemia to infections, especially pneumococcal ones, are reviewed. The pathophysiologic mechanism of functional asplenia is then discussed. The author concludes that asplenia has no predictive value in selecting children "at risk" for infection and stresses the necessity of a systematic prevention by penicillin therapy and antipneumococcal vaccination in such patients before 4 years of age.