Emile J F, Fraitag S, Andry P, Leborgne M, Lellouch-Tubiana A, Brousse N
Service d'anatomie et de cytologie pathologiques, Hôpital Necker-Enfants Malades, Paris, France.
Virchows Arch. 1995;427(2):125-9. doi: 10.1007/BF00196516.
Langerhans' cell histiocytosis (LCH) is characterized by the proliferation of large mononucleated cells containing Birbeck granules and expressing CD1a. Recent studies have demonstrated that LCH is a clonal proliferation; however, its aetiology is still unknown. Growth and differentiation of bone-marrow-derived cells are controlled by cytokines. The proliferation, differentiation and activation of normal Langerhans cells are controlled by granulocyte/macrophage colony-stimulating factor (GM-CSF) in vitro. Therefore, GM-CSF could be implicated in the pathogenesis of LCH. Indeed, LCH cells contain GM-CSF, and children with disseminated LCH have an elevated GM-CSF serum level. As a cytokine only acts on cells expressing a specific receptor, we investigated the presence of GM-CSF receptor on LCH cells. Fourteen frozen tissue samples from children with LCH were studied by in situ immunohistochemistry with two mouse monoclonal antibodies specific for the alpha chain of the GM-CSF receptor (CDw116). LCH cells of all the samples were positively stained with both antibodies. This study suggests that GM-CSF may be a growth factor for LCH cells.
朗格汉斯细胞组织细胞增多症（LCH）的特征是含有伯贝克颗粒并表达CD1a的大单核细胞增殖。最近的研究表明，LCH是一种克隆性增殖；然而，其病因仍然不明。骨髓来源细胞的生长和分化受细胞因子控制。在体外，正常朗格汉斯细胞的增殖、分化和激活受粒细胞/巨噬细胞集落刺激因子（GM-CSF）控制。因此，GM-CSF可能与LCH的发病机制有关。事实上，LCH细胞含有GM-CSF，并且播散性LCH患儿的血清GM-CSF水平升高。由于细胞因子仅作用于表达特定受体的细胞，我们研究了LCH细胞上GM-CSF受体的存在情况。用两种针对GM-CSF受体α链（CDw116）的小鼠单克隆抗体，通过原位免疫组织化学对14例LCH患儿的冷冻组织样本进行了研究。所有样本的LCH细胞均被两种抗体阳性染色。这项研究表明，GM-CSF可能是LCH细胞的生长因子。
