Immunologically induced peliosis hepatis in rats

Peliosis hepatis has been induced immunologically with anti-rat glomerular basal membrane rabbit serum in rats pre-sensitized with a rare earth metal complex, neodymium pyrocatechin disulphonate (NPD). This is the first experimental evidence that peliosis hepatis may develop as a result of an immunological process. It is noteworthy that in this experimental form of peliosis hepatis and in that observed earlier in rats treated with basic polyglutamic acid derivatives, severe defibrination was detected and, as in most human cases, not only the liver but other organs were also involved in the peliotic lesions. Since the rare earth metal compounds, among them the pyrocatechin disulphonate complex of neodymium, depress the reticulo-endothelial activity, a role of the reticulo-endothelial system in the pathogenesis of this experimental form of peliosis hepatis is suggested.     

Immunologically induced peliosis hepatis in rats.

Peliosis hepatis has been induced immunologically with anti-rat glomerular basal membrane rabbit serum in rats pre-sensitized with a rare earth metal complex, neodymium pyrocatechin disulphonate (NPD). This is the first experimental evidence that peliosis hepatis may develop as a result of an immunological process. It is noteworthy that in this experimental form of peliosis hepatis and in that observed earlier in rats treated with basic polyglutamic acid derivatives, severe defibrination was detected and, as in most human cases, not only the liver but other organs were also involved in the peliotic lesions. Since the rare earth metal compounds, among them the pyrocatechin disulphonate complex of neodymium, depress the reticulo-endothelial activity, a role of the reticulo-endothelial system in the pathogenesis of this experimental form of peliosis hepatis is suggested.     

Hepatoma and peliosis hepatis developing in a patient with Fanconi's anemia

Fanconi's anemia is a rare genetic disorder associated with congenital deformities, infections, chromosomal abnormalities, and leukemia. This brief article describes the case of a 20 year old man affected with the disease, who was given 100 mg daily of oxymetholone to cure leukopenia and thrombocytopenia. 10 months later he died of hepatic failure. Autopsy revealed hepatoma and liver peliosis, a rare condition of unknown etiology characterized by blood-filled cysts in the liver. It is possible that in the case reported here the hepatoma could have been related to the oxymetholone treatment, which conceivably could have initiated hepatic damage.     

An autopsy case of renal cell carcinoma associated with extensive peliosis hepatis.

An autopsy case of renal cell carcinoma with extensive peliosis hepatis is reported. The patient was a 34-year-old female, who had had a left nephrectomy for renal cell carcinoma but died of multiple metastases one year and 4 months after surgery, despite chemotherapy and interferon treatment. At autopsy, the liver was enlarged markedly with multiple metastatic nodules and the non-neoplastic hepatic parenchyma had a spongy appearance, due to diffusely scattered, blood-filled cystic lesions. Histological examination showed the oval to irregular shaped blood-filled spaces were lined by hepatic cell cords and located mainly in the periportal area. In addition, almost all of the sinusoids were dilated and communicated with the cystic blood-filled spaces, which also communicated directly with branches of the portal veins at various levels. Several interlobular portal branches were obstructed. The causative mechanism of peliosis hepatis has yet to be elucidated, although some causative conditions have been proposed. In this case, renal cell carcinoma may have caused the sinusoidal dilatation and the vascular changes in the portal areas, such as obstruction of terminal portal branches, may have contributed to its formation.     

Lipopeliosis: fat induced sinusoidal dilatation in transplanted liver mimicking peliosis hepatis.

A distinct peliosis-like lesion arose in the liver allograft of a 51 year old man. This lesion was caused by necrotic, fat-laden hepatocytes that released fat globules into the sinusoids. These then became strikingly distended with cysts, thus mimicking peliosis hepatitis. It is suggested that this lesion be called lipopeliosis.     

Clinical and pathological features of bacillary peliosis hepatis in association with human immunodeficiency virus infection

BACKGROUND. Peliosis hepatis is characterized by cystic, blood-filled spaces in the liver and is seen in patients with chronic infections or advanced cancer and as a consequence of therapy with anabolic steroids. Cutaneous bacillary angiomatosis is a bacterial infection that occurs in patients with human immunodeficiency virus (HIV) infection; its histologic appearance is that of a pseudoneoplastic vascular proliferation. METHODS. We studied liver tissue from eight HIV-infected patients with peliosis hepatis, two of whom also had cutaneous bacillary angiomatosis. For comparison we examined tissue from four patients who had peliosis hepatis without HIV infection. Tissues were examined histologically on routine sections and with special stains and electron microscopy. RESULTS. The histologic features seen in peliosis hepatis associated with HIV infection, but not in the four cases unrelated to HIV infection, were myxoid stroma and clumps of a granular purple material that on Warthin-Starry staining and electron microscopy proved to be bacilli. The bacilli, which could not be cultured, were morphologically identical to those found in the skin lesions of cutaneous bacillary angiomatosis. The clinical courses of two of the patients with this "bacillary peliosis hepatis" indicate that it responds to antibiotic treatment. CONCLUSIONS. HIV-associated bacillary peliosis hepatis is an unusual, treatable opportunistic infection, probably caused by the same organism that causes cutaneous bacillary angiomatosis. Our failure to find bacilli in non-HIV-associated cases implies that other pathogenetic mechanisms may also be responsible for peliosis hepatis.     

Hyperplasia and prolapse of hepatocytes into hepatic veins during longterm methyltestosteronetherapy: possible relationships of these changes to the development of peliosis hepatis and liver tumours

We report the pathological changes in liver biopsies from 10 patients (four female transexuals and six impotent males) after treatment with 150 mg methyltestosterone daily for periods of up to 3 years, and in a hemihepatectomy specimen from a female transexual who developed a liver adenoma after 37 months of this treatment. Hepatocyte hyperplasia and mild focal sinusoidal dilatation was found in most cases. In some cases there were microcysts and dissociation of hepatocytes. In nine patients there was accumulation of hepatocytes between the endothelium and the supporting collagen of hepatic veins which often resulted in either partial occlusion of their lumina or disruption of their walls. These vascular changes do not appear to have been reported before. It is postulated that a single pathological process-hyperplasia, perhaps related to the anabolic effect of methyltestosterone--could be partly responsible both for the formation of cysts through mechanical obstruction of hepatic veins and for the formation of nodules and tumours.     

Ein Fall von Peliosis hepatis

Ohne Zusammenfassung     

Peliosis hepatis with liver rupture

The case of a 50-year-old woman with a ruptured liver caused by peliosis hepatis is presented. Death occurred three days after laparotomy for an acute abdomen with a clinical diagnosis of intra-abdominal hemorrhage. Autopsy findings including histology are presented and the possible pathogenetic mechanisms are discussed. There was neither evidence of a tumor nor of drug or toxin exposure. Known etiologic factors for this disorder are discussed on the basis of the medical literature. These include consumptive primary disorders, anabolic agents and oral contraceptives. The pathogenetic sequence involves damage to the endothelial cells of the hepatic sinuses and to the reticular fiber network. This case should make physicians aware of this infrequent iatrogenic disorder which is apparently increasing in its occurrence.     

Angiogenic Process in Bacillary Angiomatosis

Eight cases of cutaneous bacillary angiomatosis related to acquired immunodeficiency syndrome were studied by light and electron microscopy and by immunohistochemistry with a panel of antibodies specific for endothelial and histiocytic markers. Light microscopy showed an inflammatory reaction with florid neovascularization and clusters of Warthin-Starry-positive bacilli. In addition, solid areas of spindle cells were also present that in some cases mimicked Kaposi's sarcoma or other sarcomas. The investigation focused primarily on the spindle cell areas and the angiogenic process present in bacillary angiomatosis. By immunohistochemistry the lesions, including the spindle cell areas, expressed all endothelial markers used; CD34, factor VIII—related antigen, and Ulex europaeus 1 were the most consistent in intensity, however. In those areas the other endothelial markers, BNH₉ and Psophocarpus tetragonolobus, were weak and not always uniform. The macrophage/monocyte markers used were α₁-antitrypsin, lysosome, kp1 (CD68), and polyclonal factor XIIIa; these revealed a sprinkle of positive cells ranging from 10% to 20% of the cell population. By electron microscopy primitive capillaries were present lined by plump endothelial cells containing frequent abluminal microprocesses forming intercellular lumina. Mitoses and intracytoplasmic lumen formation were infrequent. The study illustrates that bacillary angiomatosis is composed of active endothelial neoformation with the spindle cells representing immature endothelial cells. Furthermore, the features of this angiogenic process recapitulate the morphologic events described in experimental models.     

Partialfunktionen der Niere bei Cirrhosis hepatis

Zusammenfassung Wir untersuchten 23 Patienten mit laparoskopisch oder bioptisch gesichterter Lebercirrhose (10 Patienten ohne Ascites und 13 Patienten mit Ascites). Es wurde die Inulin- und PAH-Clearance und das tubuläre Transportmaximum für PAH bestimmt.Bei Lebercirrhosen ohne Ascites fand sich sowohl eine Verminderung mäßigen Grades der Inulin- und PAH-Clearance als auch eine Einschränkung der Nierendurchblutung. Das tubuläre Transportmaximum für PAH war normal. Bei Ascites fand sich eine deutliche Herabsetzung der Inulin-Clearance und der PAH-Clearance, eine Verminderung der Nierendurchblutung und eine signifikant erhöhte Wasserrückresorption. Das tubuläre Transportmaximum für PAH war im Bereich der Norm. Die Ursachen der Funktionseinschränkung werden diskutiert.

---

Summary 1. Inulin clearance and PAH clearance was studied in 23 patients withLaennec's cirrhosis, in 13 Patients ascites was demonstrable.2. In patients withLaennec's cirrhosis without ascites, inulin clearance and PAH clearance as well as renal blood flow was lower than normal controls. The maximal rate of tubulär transport for PAH was normal.3. In patients withLaennec's cirrhosis with ascites, inulin clearance, PAH clearance and renal blood flow was very low. Water reabsorption was significant higher than in normal controls. The maximal rate of tubular transport for PAH was normal.4. The results are discussed.