Lafon M E, Bioulac-Sage P, Longy M, Perez E, De Mascarel A, Grimaud J A, Balabaud C
Gastroenterol Clin Biol. 1987 Jun-Jul;11(6-7):510-3.
Perisinusoidal fibrosis of the liver was discovered in a 22 year-old woman with idiopathic thrombocytopenic purpura requiring splenectomy. No etiology of fibrosis could be found. As shown by immunocytochemistry, collagen types I, III, IV, laminin, and fibronectin were increased. Under electron microscopy, numerous collagen bundles and fragments of basement membrane-like material were demonstrated. Perisinusoidal cells loaded with lipids showed several of the characteristics of fibro/myofibroblasts, while the activity and number of Kupffer cells were apparently increased. The exact mechanism of fibrosis in this case remains unknown but it is likely that platelet destruction and/or Kupffer cell hyperactivity played a role.
在一名因特发性血小板减少性紫癜需行脾切除术的22岁女性患者中发现了肝窦周纤维化。未发现纤维化的病因。免疫细胞化学显示，I型、III型、IV型胶原、层粘连蛋白和纤连蛋白均增加。电子显微镜下可见大量胶原束和基底膜样物质碎片。充满脂质的肝窦周细胞表现出一些成纤维细胞/肌成纤维细胞的特征，而库普弗细胞的活性和数量明显增加。该病例中纤维化的确切机制尚不清楚，但血小板破坏和/或库普弗细胞活性亢进可能起了作用。
