and monocytes show a single red granule in the cytoplasm. Current studies suggest that there is a defective fusion protein in these individuals, which is crucial to lysosomal secretion.8 White cells in patients with Chediak-Higashi syndrome are not fully functioning and show reduced chemotaxis and bactericidal killing function. Affected children show neutropenia, albinism, and photophobia and develop recurrent infections with Staphylococcus aureus. Hepatosplenome-galy and liver failure may develop. Platelet function is affected with abnormal bleeding times and small vessel bleeding. The prognosis is poor in most children, who usually die young due to complications of infections.

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