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朗格汉斯细胞组织细胞增多症中树突状细胞标志物fascin的免疫组化表达。

Langerhans cell histiocytosis immunohistochemical expression of fascin, a dendritic cell marker.

作者信息

Pinkus Geraldine S, Lones Mark A, Matsumura Fumio, Yamashiro Shigeko, Said Jonathan W, Pinkus Jack L

机构信息

Department of Pathology, Brigham & Women's Hospital, Boston, MA 02115, USA.

出版信息

Am J Clin Pathol. 2002 Sep;118(3):335-43. doi: 10.1309/N2TW-ENRB-1N1C-DWL0.

Abstract

Langerhans cell histiocytosis (LCH) is a clonal disorder believed to be derivedfrom cells of the dendritic system. Fascin, a 55-kd actin-bundling protein, represents a highly selective marker for dendritic cells of lymphoid tissues and peripheral blood and is involved in the formation of dendritic processes in maturing epidermal Langerhans cells. Since lesional cells of LCH may represent Langerhans cells arrested at an early stage of activation, immunohistochemical expression offascin in epidermal Langerhans cells and in the lesional cells of 34 cases of LCH was evaluated in paraffin sections using an immunoalkaline phosphatase technique. Though epidermal Langerhans cells were nonreactive for fascin, lesional cells in all LCH cases exhibited immunoreactivityforfascin, CD1a, and S-100 protein. Variation in staining intensity was observed in some cases, possibly reflecting differences in cell maturation or activation. Involved tissues included bone, soft tissue, lymph node, thyroid, orbit, and extradural cranial tissue. Immunoreactivity of lesional cells of LCH for fascin supports their derivation from cells of the dendritic system and represents another alteration in the phenotype of Langerhans cells that is associated with maturation, migration, culture, or clonal expansion.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种克隆性疾病,被认为起源于树突状系统的细胞。Fascin是一种55kD的肌动蛋白捆绑蛋白,是淋巴组织和外周血中树突状细胞的高度选择性标志物,参与成熟表皮朗格汉斯细胞中树突状突起的形成。由于LCH的病变细胞可能代表处于激活早期阶段的朗格汉斯细胞,因此采用免疫碱性磷酸酶技术,在石蜡切片中评估了34例LCH患者表皮朗格汉斯细胞和病变细胞中Fascin的免疫组化表达。尽管表皮朗格汉斯细胞对Fascin无反应,但所有LCH病例的病变细胞均对Fascin、CD1a和S-100蛋白表现出免疫反应性。在某些病例中观察到染色强度的变化,这可能反映了细胞成熟或激活的差异。受累组织包括骨骼、软组织、淋巴结、甲状腺、眼眶和硬脑膜外颅组织。LCH病变细胞对Fascin的免疫反应性支持其起源于树突状系统的细胞,并代表了朗格汉斯细胞表型的另一种改变,这种改变与成熟、迁移、培养或克隆性扩增有关。

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