**Core Concept**
Chediak-Higashi syndrome (CHS) is a rare genetic disorder caused by mutations in the LYST gene, leading to a deficiency in the lysosomal trafficking regulator protein. This results in impaired lysosomal function and abnormal melanosome formation, affecting melanin production and distribution. CHS is characterized by oculocutaneous albinism, recurrent infections, and a predisposition to developing lymphoma.

**Why the Correct Answer is Right**
The defective LYST protein disrupts the normal trafficking of lysosomes and other organelles within cells, leading to their accumulation and fusion. This results in the formation of large, abnormal melanosomes that are unable to migrate to the skin and hair, causing the characteristic albinism seen in CHS patients. The impaired lysosomal function also compromises the immune system, making patients more susceptible to infections.

**Why Each Wrong Option is Incorrect**
**Option A:** CHS is primarily associated with a deficiency in the LYST gene, not a mutation in the MYD88 gene, which is involved in another type of immunodeficiency.
**Option B:** While CHS patients do experience recurrent infections, the primary cause is the impaired lysosomal function, not a deficiency in the Toll-like receptor 4 (TLR4) pathway.
**Option C:** CHS is not characterized by an increased risk of developing autoimmune disorders, but rather a predisposition to lymphoma.

**Clinical Pearl / High-Yield Fact**
CHS is an autosomal recessive disorder, and patients who are homozygous for the mutated LYST gene are at highest risk of developing clinical manifestations of the disease.

**Correct Answer:** C.