Rare Zebra

Rare disease search prototype built on Orphanet data

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ORPHA:167

Chédiak-Higashi syndrome

Also called Chédiak-Higashi disease, Chédiak-Higashi-Steinbrink syndrome

Body system
Inborn errors of metabolism
Inheritance pattern
Autosomal recessive
Typical age of onset
Childhood
Estimated prevalence
<1 / 1 000 000 (Worldwide)
Rarity class
<1 / 1 000 000

ORPHA:167 is classified under "Inborn errors of metabolism" in the Orphanet nomenclature.

Common signs

  • Hypopigmentation of the skin
  • Abnormal leukocyte morphology
  • Vacuolated lymphocytes
  • Recurrent bacterial infections
  • Recurrent infections
  • Abnormality of multiple cell lineages in the bone marrow