Alternative names: Chediak-Steinbrinck-Higashi Syndrome, CHS, Oculocutaneous Albinism with Leukocyte Defect
Chediak-Higashi syndrome is a very rare childhood disease of the immune and nervous systems that affects multiple systems of the body.
CHS damages immune system cells, leaving patients prone to repeated and persistent infections starting in infancy or early childhood. These infections tend to be very serious or life-threatening.
Only about 200 cases of this condition have been reported worldwide.
CHS is an inherited condition.
Signs and symptoms include:
There are few treatments for Chediak-Higashi syndrome. However, bone marrow transplants have been reported as being successful in some patients, especially when performed early.
If the disease is not successfully treated, most children with Chediak-Higashi syndrome reach a stage of the disorder known as the accelerated phase, thought to be triggered by a viral infection: white blood cells (which normally help fight infection) divide uncontrollably and invade many of the body's organs.
Many of the afflicted children die before age 10 from chronic infections or accelerated disease that results in lymphoma-like illness.
The accelerated phase is associated with fever, episodes of abnormal bleeding, overwhelming infections, and organ failure. These medical problems are usually life-threatening in childhood.
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