The Role of the Spleen in Sickle Cell Disease

July 04, 2009

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The Role of the Spleen in Sickle Cell Disease

by Lewis Hsu, M.D., Ph.D.
reviewed by Laura Jana, M.D., F.A.A.P.

An organ in the upper-left area of the abdomen, the spleen filters out abnormal red blood cells and helps the body's immune system fight infection. Sometimes, as in the case of sickle cell disease, red blood cells can be trapped in the spleen, a condition known as splenic sequestration. The body reacts as if it were bleeding internally because the blood trapped in the spleen cannot circulate to the heart or brain. This condition can range from mild to life threatening, depending on how much of the body's red blood cells are sequestered.

How can we detect splenic sequestration?
When red blood cells are trapped, the spleen grows tender and becomes enlarged enough to feel in the upper left part of the abdomen. Doctors detect splenic sequestration by feeling for this enlarged spleen and testing for low red-blood cell counts. You do not need a medical degree to learn how to feel for an enlarged spleen, but it does take hands-on training and practice. Your doctor can teach this when you bring in your child for a checkup.

How often should we feel for an enlarged spleen?
If your child is doing well, then just feel for the spleen several times a week just to get practice. You should feel for an enlarged spleen if your child:

Looks pale. In darker-skinned people, this may be easier to detect by looking at the lips, the inner eyelids, and the fingernail beds. Usually these areas are red or dark pink, but if they look light pink or white, then the child is pale.

Seems unusually tired.

Is unusually cranky or irritable, and perhaps has a headache. When the red blood cell count is very low, oxygen delivery to the brain may be inadequate, causing a headache.

Is sensitive to the touch in the upper-left part of her abdomen-the area above the spleen.

If you detect an enlarged spleen or if your child is displaying any of the above symptoms, you should get her in immediately for a medical evaluation. This can be an extremely serious situation and speedy evaluation and treatment may save her life.

Does splenic sequestration happen again and again?
It might. The treatment options are:

Treat each recurrence.

Start a monthly blood transfusion program to prevent recurrence of splenic sequestration.

Schedule surgery to remove the spleen, which is called an elective splenectomy.

RELATED INFORMATION
Sickle Cell Disease and Elective Splenectomy
The Immune System in Sickle Cell Disease
Sickle Cell Disease

Created February 04, 2001
Reviewed February 08, 2001

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