GIANT CYTOPLASMIC GRANULES IN LANGERHANS CELLS OF CHEDIAK-HIGASHI-SYNDROME

被引:8
作者
CARRILLOFARGA, J [1 ]
GUTIERREZPALOMERA, G [1 ]
RUIZMALDONADO, R [1 ]
ANTUNA, S [1 ]
机构
[1] INST NACL PEDIAT,DEPT DERMATOL,MEXICO CITY,MEXICO
关键词
D O I
10.1097/00000372-199002000-00012
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
Giant membrane-bound cytoplasmic granules were found in the epidermal Langerhans cells of a patient with the Chediak-Higashi syndrome. These cells also contained normal-appearing Birbeck granules. The giant granules had a granular or sometimes globular internal structure; they are believed to derive from fusion of lysosomes or some portion of Birbeck granules. It is unclear whether this morphologic change in Langerhans cells interferes with their antigen-presenting function; it may be, in part, responsible for the frequent infections seen in patients with Chediak-Higashi syndrome that are otherwise more clearly related to the abnormalities in neutrophils and lymphocytes. The Langerhans cell is another cellular type in Chediak-Higashi syndrome in which giant cytoplasmic granules are found.
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页码:81 / 87
页数:7
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