Peyrol S, Gindre D, Cordier J F, Loire R, Grimaud J A
Institut Pasteur, CNRS URA 1459, Lyon, France.
J Pathol. 1992 Dec;168(4):387-95. doi: 10.1002/path.1711680409.
Lymphangiomyomatosis (LAM) consists of smooth muscle (SM) cell proliferation of unknown origin involving the lymph nodes and the lung interstitium. From morphological studies showing both SM differentiation of the proliferating cells and lymphatic hyperplasia, hypotheses were suggested concerning the origin of the proliferation. Two cases of LAM were investigated by electron microscopy and immunohistochemistry; tissues were obtained by lymph node and open lung biopsies. Cytoplasmic and matrix protein markers were used in order to clarify the pattern of differentiation of the proliferating cells and to characterize their connective tissue environment. The proliferating cells present ultrastructural characteristics of SM cells; they contain vimentin, desmin, and alpha-SM actin and are devoid of Factor VIII, favouring a parieto-arterial origin. The connective tissue matrix inside the infiltrate is composed of interstitial collagens and basement membrane components. At the late stage of the disease, remodelling of the interstitial matrix accompanies the infiltrate and remains perilesional.
淋巴管平滑肌瘤病（LAM）由起源不明的平滑肌（SM）细胞增殖构成，累及淋巴结和肺间质。形态学研究显示增殖细胞既有SM分化又有淋巴管增生，据此提出了关于增殖起源的假说。通过电子显微镜和免疫组织化学对2例LAM进行了研究；组织通过淋巴结和开胸肺活检获取。使用细胞质和基质蛋白标志物以阐明增殖细胞的分化模式并对其结缔组织环境进行特征描述。增殖细胞呈现出SM细胞的超微结构特征；它们含有波形蛋白、结蛋白和α - SM肌动蛋白，且缺乏因子VIII，支持其源自壁动脉。浸润灶内的结缔组织基质由间质胶原和基底膜成分组成。在疾病晚期，间质基质重塑伴随浸润灶出现并局限于病灶周围。
