Zimmermann A, Diem P, Cottier H
Virchows Arch A Pathol Anat Histol. 1982;396(2):187-95. doi: 10.1007/BF00431240.
The so-called "histiocytoid cardiomyopathy" is an unusual cardiac disorder of infancy and childhood, characterized by the presence of numerous foamy, lipid-containing cells between the endocardium and the striated myocardial cells of the left ventricle and the interventricular septum. The disease usually affects females, the clinical picture being dominated by severe disturbances of conduction. The original designations of the disorder stem from the morphological resemblance of the foamy cells to lipid-laden histiocytes. However, subsequent investigations have shown these cells to contain myofibrils interposed with Z lines. It has, therefore, been suspected that the leading cell population might be related to the myocardium. Using a histochemical method for the demonstration of cholinesterase activity in the foamy cells, we present evidence that "histiocytoid" cardiomyopathy may in fact correspond to a maldevelopment of the Purkinje cell system of the heart.
所谓的“组织细胞样心肌病”是婴幼儿期一种罕见的心脏疾病，其特征是在左心室和室间隔的心内膜与横纹肌心肌细胞之间存在大量含脂质的泡沫细胞。该病通常影响女性，临床表现以严重的传导障碍为主。该疾病最初的命名源于泡沫细胞与富含脂质的组织细胞在形态上的相似性。然而，随后的研究表明这些细胞含有插入Z线的肌原纤维。因此，有人怀疑主要的细胞群可能与心肌有关。通过一种组织化学方法来显示泡沫细胞中的胆碱酯酶活性，我们提供证据表明“组织细胞样”心肌病实际上可能对应于心脏浦肯野细胞系统的发育异常。
