Chediak higashi Syndrome: NExt oral pathology
Chediak-Higashi syndrome (CHS) and Döhle bodies are two distinct entities, but they are both related to abnormalities in white blood cells. Let's explore each in more detail: **1. Chediak-Higashi Syndrome (CHS):** - **Genetic Basis:** CHS is a rare autosomal recessive genetic disorder caused by mutations in the LYST gene, affecting lysosomal function. - **Clinical Features:** - Partial albinism: Individuals with CHS often have pale skin, light hair, and light-colored eyes due to reduced pigmentation. - Immune system dysfunction: CHS leads to recurrent bacterial infections due to impaired neutrophil function. - Giant granules: Abnormal giant granules are present in various white blood cells, including granulocytes, monocytes, and lymphocytes. - Neurological involvement: Some individuals may experience progressive neurological deterioration. - **Ocular Abnormalities:** Nystagmus, strabismus, and photophobia may be present. - **Hematological Manifestations:** Anemia and b