immune disorders
Chédiak Higashi Syndrome
Introduction
Chédiak-Higashi syndrome is a very rare hereditary immunodeficiency disorder characterized by recurrent bacterial respiratory and other infections and lack of pigment in the hair, eyes, and skin (albinism). People with Chédiak-Higashi syndrome usually have pale skin, light-colored or white hair, and pink or pale blue-gray eyes. Doctors examine a sample of blood to check for abnormalities and do genetic tests to confirm the diagnosis. Treatment involves antibiotics to prevent infections, other drugs to help the immune system function better, and, if possible, stem cell transplantation. (See also Overview of Immunodeficiency Disorders .) Chédiak-Higashi syndrome is a Primary immunodeficiency . It is usually inherited as an People with Chédiak-Higashi syndrome are more susceptible to infections because phagocytes (types of Components of the Immune System , including neutrophils, eosinophils, monocytes, and macrophages) do not function normally. Phagocytes are cells that ingest and kill microorganisms. 