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组织细胞性髓性网状细胞增生症的尸检及超微结构发现

Necropsy and ultrastructural findings in histiocytic medullary reticulosis.

作者信息

Piñol-Aguadé J, Ferrando J, Tomás J M, Mieras C, Peyrí J

出版信息

Br J Dermatol. 1976 Jul;95(1):35-44. doi: 10.1111/j.1365-2133.1976.tb15533.x.

DOI:10.1111/j.1365-2133.1976.tb15533.x
PMID:952738
Abstract

In a case of histiocytic medullary reticulosis with protracted course and necrotic skin lesions, histological study revealed infiltration of the fat tissue by atypical reticulohistiocytic cells, areas of associated fat necrosis and vascular lesions. Autophagocytosis (phagocytosis of erythrocytes, erythroblasts, platelets, lymphoid cells, and nuclear debris) was found in biopsy specimens of the subcutaneous infiltrates, lymph nodes, and bone marrow, and in necropsy specimens of skin, iliac mesenteric lymph nodes, bone marrow, liver, spleen, and suprarenal glands. Electron microscopy of various viscera showed abnormal histiocytic cells containing multiple phagocytosed erythrocytes and other blood cells in different stages of destruction and digestion. Multi-membranous bodies also were present. Defective lysosomal degradation of intracytoplasmatic ceroids in this condition is postulated.

摘要

在一例病程迁延且伴有皮肤坏死病变的组织细胞性髓性网状细胞增生症中,组织学研究显示非典型网状组织细胞浸润脂肪组织,伴有脂肪坏死区域和血管病变。在皮下浸润灶、淋巴结和骨髓的活检标本以及皮肤、髂肠系膜淋巴结、骨髓、肝脏、脾脏和肾上腺的尸检标本中发现了自噬现象(吞噬红细胞、成红细胞、血小板、淋巴细胞和核碎片)。对各种内脏的电子显微镜检查显示,异常组织细胞含有多个吞噬的红细胞和处于不同破坏和消化阶段的其他血细胞。还存在多膜体。推测在这种情况下,细胞质内类蜡质的溶酶体降解存在缺陷。

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