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Chediak-Higashi syndrome

Treatment:

There is no specific treatment for Chediak-Higashi syndrome. Bone marrow transplants appear to have been successful in several patients, especially when performed early in the disease.

Antibiotics are used to treat infections. Antiviral drugs such as acyclovir and chemotherapy drugs are often used in the accelerated phase of the disease. Surgery may be needed to drain abscesses in some cases.

Support Groups:

Chediak-Higashi Syndrome Association -- www.chediak-higashi.org

Expectations (prognosis):

Death often occurs in the first 10 years of life because of chronic infections or accelerated disease that results in lymphoma-like illness. However, persons have survived for longer.

Complications:

Frequent infections especially with Epstein-Barr virus
Lymphoma-like cancer
Early death

Calling your health care provider:

Call your health care provider if you have a family history of this disorder and you are planning to have children.

Call for an appointment with your health care provider if your child shows symptoms of Chediak-Higashi syndrome.

Review Date: 7/1/2007
Reviewed By: Brian Kirmse, MD, Mount Sinai School of Medicine, Department of HumanGenetics, New York, NY. Review provided by VeriMed Healthcare Network.

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