Mazal P R, Hainfellner J A, Preiser J, Czech T, Simonitsch I, Radaszkiewicz T, Budka H
Institute of Neurology, University of Vienna, Austria.
Clin Neuropathol. 1996 Mar-Apr;15(2):87-91.
The immunophenotype of 6 cases of Langerhans cell histiocytosis (LCH) of the hypothalamus and 3 cases of cranial bone manifestation of LCH was investigated by means of immunohistochemistry on paraffin sections. Antibodies against S 100 protein, lysozyme, CD68 (PG-M1), CD68 (KP1), HLA-DR, beta 2 microglobulin, placental alkaline phosphatase (PLAP), the monoclonal antibody MAC 387, and a monoclonal antibody against CD1a were used. All examined cases showed positive staining of lesional cells for S 100 protein, HLA-DR, beta 2 microglobulin, macrophage associated markers and CD1a. According to the "confidence levels" of the Writing Group of the Histiocyte Society [Chu et al. 1987], a "definite diagnosis" of LCH requires the demonstration either of Birbeck granules in lesional cells by electron microscopy, or of CD1a antigenic determinants on the surface of lesional cells. Since electron microscopy of these rare CNS lesions is not possible in many cases, we are now able to give a definite diagnosis of LCH of the hypothalamus by means of immunohistochemistry for CD1 a on routinely fixed and processed tissue.
采用石蜡切片免疫组化方法，对6例下丘脑朗格汉斯细胞组织细胞增生症（LCH）及3例LCH颅骨表现病例的免疫表型进行了研究。使用了抗S 100蛋白、溶菌酶、CD68（PG - M1）、CD68（KP1）、HLA - DR、β2微球蛋白、胎盘碱性磷酸酶（PLAP）的抗体、单克隆抗体MAC 387以及抗CD1a单克隆抗体。所有检查病例的病变细胞对S 100蛋白、HLA - DR、β2微球蛋白、巨噬细胞相关标志物及CD1a均呈阳性染色。根据组织细胞协会写作组的“置信水平”[Chu等人，1987年]，LCH的“明确诊断”需要通过电子显微镜在病变细胞中显示伯贝克颗粒，或在病变细胞表面显示CD1a抗原决定簇。由于在许多情况下无法对这些罕见的中枢神经系统病变进行电子显微镜检查，我们现在能够通过对常规固定和处理的组织进行CD1a免疫组化来明确诊断下丘脑LCH。
