Prognosis
The most common cause of death in patients with Chediak Higashi syndrome results from recurrent infections or the development of an accelerated phase where there is lymphoproliferation into major organs. 80% of deaths occur in the first decade of life, and those who survive into adulthood develop progressive neurological symptoms.
Death in childhood is usually due to overwhelming infection.
Reference
- Anitha Ajitkumar et al. Chediak-Higashi Syndrome. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): January 2025
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