This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Pathology
[{'content': 'Defective phagolysosome formation is a feature of ___________. \nSelect and explain. \nA. Chronic granulomatus disease, B. Chediak-Higashi syndrome, C. Fanconi Anemia, D. Ataxia telangiectasia', 'role': 'user'}, {'content': 'Answer: B \nExplanation: Chediak-Higashi\xa0 syndrom:\n\nChediak-Higashi\xa0 syndrome\xa0 is\xa0 characterized\xa0 by\xa0 defective fusion of phagosomes and lysosomes, resulting in defective phagocyte function and susceptibility to infections.\xa0\nThe\xa0 main\xa0 leukocyte\xa0 abnormalities\xa0 are\xa0 neutropenia, defective degranulation, and delayed microbial killing.\xa0\nThe\xa0 affected\xa0 leukocytes\xa0 contain\xa0 giant\xa0 granules,\xa0 which are\xa0 readily\xa0 seen\xa0 in\xa0 peripheral\xa0 blood\xa0 smears\xa0 and\xa0 are\xa0thought to result from aberrant phagolysosome fusion.\nIn\xa0 addition,\xa0 there\xa0 are\xa0 abnormalities\xa0 in\xa0 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.\n\nReference-Robbins BASIC PATHOLOGY 10th edition pg-172', 'role': 'assistant'}]