Defective phagolysosome formation is a feature of ___________.
Correct Answer: Chediak-Higashi syndrome
Description: Chediak-Higashi syndrom:
Chediak-Higashi syndrome is characterized by defective fusion of phagosomes and lysosomes, resulting in defective phagocyte function and susceptibility to infections.
The main leukocyte abnormalities are neutropenia, defective degranulation, and delayed microbial killing.
The affected leukocytes contain giant granules, which are readily seen in peripheral blood smears and are thought to result from aberrant phagolysosome fusion.
In addition, there are abnormalities in melanocytes (leading to albinism), cells of the nervous system (associated with nerve defects), and platelets (causing bleeding disorders) The gene associated with this disorder encodes a large cytosolic protein called LYST, which is believed to regulate lysosomal trafficking.
Reference-Robbins BASIC PATHOLOGY 10th edition pg-172
Category:
Pathology
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