Lafon M E, Bioulac-Sage P, Balabaud C
Laboratoire des Interactions Cellulaires, Université de Bordeaux II, France.
Liver. 1988 Feb;8(1):24-7. doi: 10.1111/j.1600-0676.1988.tb00962.x.
Three patients showing all the symptoms of idiopathic thrombocytopenic purpura underwent a splenectomy. A wedge liver biopsy revealed hepatic fibrosis around central veins and an increased perisinusoidal network on Sirius red staining. Fibrosis was moderate or mild. Liver histology was otherwise normal, as were liver function tests. Hepatic fibrosis could not be attributed to any known causes. Electron microscopy showed numerous Kupffer cells with intense phagocytic activity, and perisinusoidal cells with some of the characteristics of fibro/myofibroblasts. The mechanisms of fibrosis remain unknown but could be attributed, by analogy to agnogenic myeloid metaplasia, to the massive destruction of platelets liberating PDGF and to increased activation of macrophages.
三名表现出特发性血小板减少性紫癜所有症状的患者接受了脾切除术。楔形肝活检显示中央静脉周围肝纤维化,天狼星红染色显示肝血窦周围网络增加。纤维化程度为中度或轻度。肝脏组织学其他方面正常,肝功能检查也正常。肝纤维化无法归因于任何已知原因。电子显微镜显示大量具有强烈吞噬活性的库普弗细胞,以及具有一些成纤维细胞/肌成纤维细胞特征的肝血窦周围细胞。纤维化的机制尚不清楚,但类似于原因不明的髓样化生,可能归因于血小板的大量破坏释放血小板衍生生长因子以及巨噬细胞激活增加。