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赫尔曼斯基-普德拉克综合征:骨髓巨噬细胞的超微结构

The Hermansky-Pudlak syndrome: ultrastructure of bone marrow macrophages.

作者信息

White J G, Witkop C J, Gerritsen S M

出版信息

Am J Pathol. 1973 Mar;70(3):329-43.

PMID:4347622
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1904019/
Abstract

The present investigation has explored the fine structure of the lipid inclusions which fill the bone marrow macrophages of patients with the Hermansky-Pudlak syndrome. Red blood cells are the major substrate of the reticular macrophages, and incomplete digestion of erythrocytes leads to formation of the massive inclusions. Progressive transformation of the macrophages results in an end-stage cell whose damaged cytoplasm is nearly replaced by huge lipid-containing vacuoles surrounded by particulate debris. This type of cell has not been found in bone marrows from patients with hemolytic, thrombocytopenic or lipid storage diseases.

摘要

本研究探索了赫尔曼斯基-普德拉克综合征患者骨髓巨噬细胞中脂质包涵体的精细结构。红细胞是网状巨噬细胞的主要底物,红细胞消化不完全会导致大量包涵体的形成。巨噬细胞的渐进性转变会产生一种终末期细胞,其受损的细胞质几乎被由颗粒碎片包围的巨大含脂空泡所取代。在溶血性、血小板减少性或脂质贮积病患者的骨髓中未发现这种类型的细胞。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/04f9a7411009/amjpathol00259-0074-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/5af64a82e433/amjpathol00259-0075-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/f7e822acefda/amjpathol00259-0076-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/855ae7db4941/amjpathol00259-0076-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/a8a62f1e43d8/amjpathol00259-0077-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/52858698ce52/amjpathol00259-0077-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/af7d29b6b0e3/amjpathol00259-0073-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/f3e29b503271/amjpathol00259-0073-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/04f9a7411009/amjpathol00259-0074-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/5af64a82e433/amjpathol00259-0075-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/f7e822acefda/amjpathol00259-0076-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/855ae7db4941/amjpathol00259-0076-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/a8a62f1e43d8/amjpathol00259-0077-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/52858698ce52/amjpathol00259-0077-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/af7d29b6b0e3/amjpathol00259-0073-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/f3e29b503271/amjpathol00259-0073-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1b3/1904019/04f9a7411009/amjpathol00259-0074-a.jpg

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Int Ophthalmol. 1981 Aug;4(1-2):113-22. doi: 10.1007/BF00139585.
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A CLINICAL, BIOCHEMICAL AND ELECTRON MICROSCOPIC STUDY OF LATE INFANTILE AMAUROTIC FAMILY IDIOCY.晚发性婴儿黑蒙性家族性白痴的临床、生化及电子显微镜研究
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