Perisinusoidal fibrosis of the liver in patients with thrombocytopenic purpura.
10 patients with thrombocytopenic purpura (TP) underwent splenectomy. Eight of these patients had idiopathic TP (certain or probable). All had normal liver function tests. Liver histology of the surgical biopsy was normal with the exception of a non specific mild portal infiltration in 6 cases. On Sirius red staining the perisinusoidal network was normal in 3 cases, mildly or moderately increased in 5 cases and often associated with perivenular fibrosis. Collagen types I, III, IV, laminin and fibronectin were increased in the 8 biopsies tested. On semi-thin sections, numerous Kupffer cells were observed. Under the electron microscope, sinusoidal abnormalities were very similar in all 7 patients studied: numerous Kupffer cells containing abundant lysosomes, numerous collagen bundles in the Disse space, active endothelial cells, transformation of some perisinusoidal cells into cells with some of the characteristics of fibroblasts (increased RER) and myofibroblasts (peripheral condensations of the filamentous network), increased fragments of basement membrane-like material. In two cases there was an increase in the number of perisinusoidal cells loaded with lipids. The similarity of the lesions and the absence of other fibrogenic causes (except in 2 cases) suggest that TP may represent another group of diseases with perisinusoidal fibrosis. The aetiology of fibrosis remains unknown but platelet derived growth factor and activated macrophages may play a major role.
D000293:Adolescent; D000328:Adult; D003094:Collagen / Q000032:analysis; D005260:Female; D006801:Humans; D007728:Kupffer Cells / Q000473:pathology; D008099:Liver / Q000473:pathology / Q000648:ultrastructure; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010978:Platelet Factor 4 / Q000502:physiology; D010982:Platelet-Derived Growth Factor / Q000502:physiology; D011696:Purpura, Thrombocytopenic / Q000473:pathology*
D010982:Platelet-Derived Growth Factor; D010978:Platelet Factor 4; D003094:Collagen