Pediatric White Cell Disorders

Neutropenia

About 70 percent of white blood cells in the human body are neutrophils, white blood cells that fight fungi and bacteria. Neutrophils mature in the bone marrow for about two weeks before being released to fight infection in the body. They circulate for about six hours, searching for foreign substances to attack.

Neutropenia (a decrease in neutrophils) can be caused by a problem in the bone marrow that keeps new cells from being formed, or by a condition that prematurely destroys cells in the bloodstream, such as autoimmune neutropenia. Medicine for unrelated medical disorders, other blood cell deficiencies, hereditary diseases, cancers, tuberculosis, vitamin deficiencies, and other conditions can cause neutropenia. If the neutropenia is caused by a viral infection, it goes away when the infection is cured. A condition called chronic benign neutropenia of childhood also usually clears up by itself. Congenital neutropenia is sometimes referred to as Kostmann's syndrome.

Treatment for neutropenia depends upon its cause. If the condition does not clear up by itself, the patient may need drugs or other therapies. Drugs typically given include growth factors such as G-CSF (granulocyte colony-stimulating factor) or GM-CSF (granulocyte-macrophage colony-stimulating factor). Autoimmune reactions may be treated by corticosteroids or immunosuppressive therapies. In some cases an enlarged spleen may be surgically removed if it is found to be trapping white blood cells. In rare cases a bone marrow transplant may be appropriate.