Do adults with sickle cell disease get splenic sequestration?

Answer 1

Yes, it is one of the major reasons for death

The condition called splenic sequestration is when blood builds up in the spleen. As the blood builds up it makes the belly grow and become rigid with the build up. The normal way to treat it is with blood transfusions and hospital care. It can happen in children with sickle cell disease because the sickle shaped blood cells can get trapped easily in the spleen. It is rare for this condition to happen in adults with sickle cell anemia.

According to: http://sickle.bwh.harvard.edu/spleen.html
"Acute exacerbation of anemia in the patient with sickle cell disease is a significant cause of morbidity and mortality. The most common processes leading to these "crises" are acute splenic sequestration and transient erythroid hypoplasia.

Acute splenic sequestration is caused by intrasplenic trapping of red cells causing a precipitous fall in hemoglobin level and the potential for hypovolemic shock. With the recent decline in mortality from pneumococcal sepsis, ASSC has become a leading cause of death in children with sickle cell disease."

Check out Wikipedia: http://en.wikipedia.org/wiki/Sickle_cell_disease
"Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anemia. This autosplenectomy increases the risk of infection from encapsulated organisms; preventive antibiotics and vaccinations are recommended for those with such asplenia. Liver failure may also occur with time.

Splenic sequestration crisis. An acute, painful enlargement of the spleen. The abdomen becomes bloated and very hard. Management is supportive, sometimes with blood transfusion."

Check out this site: http://www.doh.wa.gov/ehsphl/phl/newborn/splcris.htm
"WHAT IS SPLENIC SEQUESTRATION? (SPLEEN CRISIS)
When sickled cells block the blood vessels leading out of the spleen, blood stays in the spleen instead of flowing through it. This causes the spleen to get bigger. When this happens the blood count (hemoglobin and hematocrit) falls and the spleen gets very large and easy to feel. This is called splenic sequestration crisis (or "spleen crisis"). Splenic sequestration can sometimes be painful."

The rest of the article can prove very useful.

According to: http://www.medscape.com/viewarticle/475043
"Acute splenic sequestration crisis (ASSC) is a common complication of sickle cell anemia in children. ASSC is generally not seen in adults with the SS genotype but occasionally can be seen in adults with the SC genotype. We present a case of fulminant ASSC in an adult with hemoglobin SC who developed high fever, intense abdominal pain, leukocytosis, and jaundice."