Lau Sean K, Chu Peiguo G, Weiss Lawrence M
Department of Pathology, City of Hope National Medical Center, Duarte, CA 91010, USA.
Am J Surg Pathol. 2008 Apr;32(4):615-9. doi: 10.1097/PAS.0b013e31815b212b.
Langerin is a type II transmembrane C-type lectin associated with the formation of Birbeck granules in Langerhans cells. Langerin is a highly selective marker for Langerhans cells and the lesional cells of Langerhans cell histiocytosis. Although Langerin protein expression in Langerhans cell histiocytosis has been previously documented, the specificity of Langerin expression as determined by immunohistochemistry in the context of other histiocytic disorders has not been well established. In the present study, Langerin immunoreactivity was examined in a series of histiocytic disorders of monocyte/macrophage and dendritic cell derivation to assess the specificity and utility of Langerin as a diagnostic marker for Langerhans cell histiocytosis. Immunohistochemical expression of CD1a was also evaluated for comparison. Seventeen cases of Langerhans cell histiocytosis and 64 cases of non-Langerhans cell histiocytic disorders were examined. Langerin and CD1a were uniformly expressed in all cases of Langerhans cell histiocytosis, with the exception of one case that was positive for Langerin and negative for CD1a. Among the non-Langerhans cell histiocytic disorders evaluated, focal Langerin immunoreactivity was observed only in 2 of 10 cases of histiocytic sarcoma. All non-Langerhans cell histiocytic disorders showed no expression of CD1a. Langerin expression seems to be a highly sensitive and relatively specific marker of Langerhans cell histiocytosis. Immunohistochemical evaluation of Langerin expression may have utility in substantiating a diagnosis of Langerhans cell histiocytosis and separating this disorder from other non-Langerhans cell histiocytic proliferations.
朗格素是一种II型跨膜C型凝集素，与朗格汉斯细胞中伯贝克颗粒的形成有关。朗格素是朗格汉斯细胞以及朗格汉斯细胞组织细胞增多症病灶细胞的高度特异性标志物。尽管此前已有文献记载朗格汉斯细胞组织细胞增多症中朗格素蛋白的表达情况，但在其他组织细胞疾病背景下，通过免疫组织化学确定的朗格素表达特异性尚未得到充分证实。在本研究中，我们检测了一系列源自单核细胞/巨噬细胞和树突状细胞的组织细胞疾病中的朗格素免疫反应性，以评估朗格素作为朗格汉斯细胞组织细胞增多症诊断标志物的特异性和实用性。还评估了CD1a的免疫组织化学表达以作比较。我们检查了17例朗格汉斯细胞组织细胞增多症病例和64例非朗格汉斯细胞组织细胞疾病病例。除1例朗格素阳性而CD1a阴性的病例外，朗格素和CD1a在所有朗格汉斯细胞组织细胞增多症病例中均呈一致表达。在评估的非朗格汉斯细胞组织细胞疾病中，仅在10例组织细胞肉瘤中的2例中观察到局灶性朗格素免疫反应性。所有非朗格汉斯细胞组织细胞疾病均未显示CD1a表达。朗格素表达似乎是朗格汉斯细胞组织细胞增多症的高度敏感且相对特异的标志物。朗格素表达的免疫组织化学评估可能有助于证实朗格汉斯细胞组织细胞增多症的诊断，并将该疾病与其他非朗格汉斯细胞组织细胞增殖区分开来。
