Hughes D, Buckley P J
Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06510.
Am J Surg Pathol. 1993 May;17(5):482-90. doi: 10.1097/00000478-199305000-00007.
Idiopathic retroperitoneal fibrosis (IRF) is an uncommon disease of obscure etiology and pathobiology. Using sections of frozen and paraffin-embedded tissue, an immunohistochemical technique, and antibodies to a variety of macrophage- and lymphocyte-associated antigens, we studied six examples of IRF. The results showed a large population of spindle-shaped cells that expressed the immunophenotype of a tissue macrophage, that is, Leu 3a,b (CD4)+, MY7 (CD13)+, Leu M5 (CD11c)+, KP-1 and EBM-11 (CD68)+, human leukocyte antigen (HLA-DR)+, leukocyte common antigen (CD45)+, HAM-56+, and MAC387+. A subpopulation of these cells also reacted with an antibody to the "activation" antigen, interleukin 2R (CD25). A control group of "fibroblastic" lesions including keloids, desmoid tumors, and an aggressive fibromatosis displayed minimal reactivity with this panel of antibodies. The abundance of macrophages suggests that they may play an important role in the pathogenesis of IRF. If, as has been suggested by some studies, IRF is an immune-mediated phenomenon, the macrophages may be triggered to produce cytokines that stimulate fibroblast proliferation and subsequent fibrosis that characterize this disease.
特发性腹膜后纤维化（IRF）是一种病因和病理生物学不明的罕见疾病。我们使用冷冻和石蜡包埋组织切片、免疫组织化学技术以及针对多种巨噬细胞和淋巴细胞相关抗原的抗体，对6例IRF进行了研究。结果显示大量梭形细胞表达组织巨噬细胞的免疫表型，即Leu 3a,b（CD4）+、MY7（CD13）+、Leu M5（CD11c）+、KP-1和EBM-11（CD68）+、人类白细胞抗原（HLA-DR）+、白细胞共同抗原（CD45）+、HAM-56+和MAC387+。这些细胞的一个亚群也与针对“活化”抗原白细胞介素2R（CD25）的抗体发生反应。包括瘢痕疙瘩、硬纤维瘤和侵袭性纤维瘤病在内的“纤维母细胞性”病变对照组对该组抗体的反应极小。巨噬细胞的大量存在表明它们可能在IRF的发病机制中起重要作用。如果如一些研究所表明的，IRF是一种免疫介导的现象，巨噬细胞可能被触发产生细胞因子，刺激成纤维细胞增殖以及随后出现该疾病所特有的纤维化。
