Chediak-Higashi syndrome

Chédiak-Higashi syndrome is a rare childhood autosomal recessive disorder that affects multiple systems of the body, which arises from a mutationthat causes silvery hair. It is a disease with impaired leukocyte function due to failure of phagolysosome formation. As a result of disordered intracellular trafficking there is impaired lysosome degranulation with phagosomes. In addition secretion of lytic secretory granules by cytotoxic T cells is also affected. The disease is characterised by large lysosome vesicles in phagocytes who have poor bactericidal function leading susceptibility to infections, abnormalities in nuclear structure of leukocytes, anaemia, hepatomegaly.

Chédiak-Higashi like syndrome, (also known as Griscelli syndrome, Silvery hair syndrome, or Albinism partial immunodeficiency) is a similar rare childhood autosomal recessive disorder that causes hypopigmentation of the skin and the hair, and can cause a fatal hemophagocytic syndrome, for which the only cure is bone-marrow transplantation.

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It uses material from the http://en.wikipedia.org/wiki/Chediak-Higashi+syndrome Wikipedia article Chediak-Higashi syndrome.