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.     

Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura

Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months.     

Peliosis hepatis associated with lymphoplasmacytic lymphoma: an autopsy case report

A 72-year-old man with no previous history of liver disease was admitted to our university hospital with severe dyspnea, edema of the lower limbs, and weight loss. Within a few days of hospitalization, he died due to severe bleeding in the upper digestive tract. At autopsy, the liver displayed typical gross features of peliosis hepatis. In addition, a diffuse infiltration of liver, spleen, bone marrow, and lymph nodes by lymphoplasmacytic lymphoma was disclosed by light microscopy. In the liver, the neoplastic cells partially filled the peliotic cavities. Peliosis hepatis is a rare liver disease characterized by multiple blood-filled, dilated cavities within the liver parenchyma. Association of lymphoplasmacytic lymphoma and peliosis hepatis has rarely been reported in the literature. The pathologic findings of such an unusual association and a review of the literature are presented.     

Murine Model of Bartonella henselae Infection in the Immunocompetent Host

Bartonella henselae is an emerging pathogen causing cat scratch disease, bacillary angiomatosis, and peliosis hepatis. Progress in understanding the pathogenesis of and the immune response to these infections has been limited by the lack of an animal model. Following intraperitoneal infection of C57BL/6 mice with B. henselae, organs were cleared of cultivatable bacteria within 6 days. In contrast, B. henselae DNA could be detected in liver tissue for at least 3 months. Liver tissue showed granulomatous inflammation reaching its highest degree of intensity during the fourth week of infection and resolving within 12 weeks postinfection. This mouse model is applicable to the study of the pathogenesis of B. henselae and the immune response to this pathogen in the immunocompetent host.     

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.     

Peliosis hepatis. Twelve cases associated with oral androgen therapy

Peliosis hepatis was encountered in 12 patients treated with high-dose oxymetholone or fluoxymesterone therapy. In three cases liver failure was the primary cause of death. In one case, the diagnosis was established by biopsy, the androgen therapy was discontinued, and the lesion was absent at autopsy two years later. In eight cases peliosis hepatis was an incidental finding at postmortem examination. The clinical and anatomic features of these cases are described, and previously reported cases of peliosis are briefly reviewed. A hypothesis is offered to explain the association of this peculiar lesion with anabolic androgen therapy.     

"Have you seen this?" peliosis hepatis

Peliosis hepatis, characterized by the presence of blood-filled spaces within hepatic parenchyma, developed in C57Bl mice implanted subcutaneously with melanoma cells 23 days previously. The peliosis was associated with dilated hepatic sinusoids that were lined by prominent, proliferating endothelial cells. The development of peliosis hepatis was completely abrogated when melanoma growth was inhibited by administration of dexamethasone. These features support the concept that peliosis hepatis can be induced by a circulating tumor-derived endothelial growth factor such as vascular endothelial growth factor.     

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.     

PELIOSIS HEPATIS

So-called "Fumyaku-han" of the bovine liver was examined histologically. Localized hemorrhagic foci in the liver have turned out to be blood cavities lined with endothelium associated with hepatic cells. There was no sign of hepatic cell necrosis. The liver lesion was thus identified as phlebectatic type of peliosis hepatis based on histological characteristics and in the light of the literature.     

High Incidence of Peliosis Hepatis in Autopsy Cases of Aplastic Anemia With Special Reference to Anabolic Steroid Therapy

The livers were collected from 47 autopsy cases of aplastic anemia aiming to study the correlation between the use of androgenic anabolic steroid and the formation of peliosis hepatis. Association of peliosis hepatis was found in 7 cases out of 19 cases with anabolic steroid therapy, while peliotic cavities were found only in one case out of the remaining 28 cases without anabolic steroid therapy. Out of 7 cases specified above, peliotic cavities in 5 cases were phlebectatic; phlebectatic and parenchymal types of peliotic cavities coexisted in one case, and one case was parenchymatous. Besides these cavity formations, remarkable dilatation of sinusoids often distinct at the periphery of the hepatic lobule was found in the 7 cases.     

Report of Nine Cases

Nine cases of peliosis hepatis are reported; five of these were associated with the administration of androgen or anabolic androgenic steroids and a sixth with large doses of medroxy-progesterone acetate. In five instances, neoplasm was present as an underlying disease. Antemortem evidence of liver disease was detected in six of seven cases but was not severe and had not contributed to death. The pathogenesis of blood-filled cystic cavities is discussed and the literature reviewed. Angiopathy of hepatic sinusoida in patients with wasting diseases or in those receiving androgens in coexistence with passive congestion of the liver appear to be factors in the pathogenesis of pleiosis hepatis. ACTA PATH. JAP. 29 : 457–469, 1979.     

Effects of Ethyl Carbamate and its Metabolites on the Antibody Response in Splenocyte Cults from Female Balb/C Mice

To investigate a possible role by cytochrome P450 (P450) in ethyl carbamate-induced immunosuppression, an attempt to assess the ability of ethyl carbamate, its metabolites produced by P450 (i.e., ethyl N-hydroxycarbamate and vinyl carbamate), and methyl carbamate to suppress the polyclonal antibody response induced by bacterial lipopolysaccharide was made in splenocyte cultures isolated from female Balb/C mice. The results showed that vinyl carbamate and ethyl N-hydroxycarbamate were more immunosuppressive compared to ethyl carbamate. A structurally related analogue, methyl carbamate, did not suppress the antibody response. These results indicate that metabolism of ethyl carbamate by P450 may produce more immunosuppressive metabolites as in ethyl carbamate-induced carcinogenicity. A pre-incubation study with phenobarbital-induced liver microsomes in the presence of NADPH-generating system showed that the antibody response was suppressed by ethyl carbamate when splenocytes were pre-incubated with ethyl carbamate and microsomes simultaneously. Moreover, the suppression was completely recovered by the addition of a P450 inhibitor, aminoacetonitrile, in the pre-incubation. Taken together, the present results indicate that metabolism of ethyl carbamate by P450 enzyme(s) may be an important pathway to cause immunosuppression.     

Peliosis-like ultrastructural changes of the hepatic sinusoids in human chronic hypervitaminosis A: Report of three cases

In addition to hypertrophy of Ito cells and perisinusoidal fibrosis, previously unrecognized ultrastructural abnormalities of the hepatic sinusoids were observed in three patients with chronic hypervitaminosis A: 1) large areas of communication between the sinusoidal lumina and the perisinusoidal spaces, allowing extravasation of blood cells; 2) marked dilation of the perisinusoidal spaces; and 3) swelling and clarification of endothelial cells. Most of these changes, along with some other sinusoidal barrier alterations previously reported in chronic hypervitaminosis A (i.e., bleb formation on the sinusoidal membrane of the hepatocytes and the presence of multiple cellular layers lining the sinusoids), are strikingly similar to those observed in peliosis hepatis. The present findings suggest that sinusoidal barrier abnormalities might constitute a major event in the pathophysiology of vitamin A-induced liver injury as well as of peliosis hepatis.     

PELIOSIS HEPATIS Report of Two Autopsy Gases with a Review of Literature

Two cases of peliosis hepatis, treated with a large dose of predonine for aplastic anemia and multiple myeloma, respectively, were reported. Case 1 showed no abnormal liver function, but, in Case 2 hepatomegaly, retention of ICG, and elevation of LDH level despite of normal serum transaminase were noted. In both cases, peliotic lesions were incidentally observed at autopsy. In Case 1, the lesion was localized in the right lobe only, but, in Case 2 the lesion was present throughout the entire lobes of the liver involving also a part of the spleen and bone marrow of the sternum. Innumerable blood-filled cavities were surrounded by incomplete reticulin or injured hepatocytes. In smaller lesions observed in Case 1, focal necrosis, hemorrhage, inflammation, and thrombi were prominent features. On the other hand, in extensive lesions, as in Case 2, a marked liver cell dissociation with sequent irregular reticulin arrangement was present. Though the etiology of peliosis hepatis is still uncertain in these cases, it was assumed that a large dose of predonine might have some correlation to the occurrence.     

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 nonneoplastic 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.     

胆道闭锁肝内外胆系组织病理形态学分析

目的 通过胆道闭锁（BA）肝门纤维块、肝脏组织的病理及其超微结构观察，对肝门成纤维细胞分化程度进行评分，并与肝纤维化分级进行相关分析。方法 选取BA患儿作为研究对象，术中取肝门纤维块及肝脏组织标本；研究同期选取疑似BA经术中胆道造影除外BA，诊断为胆汁淤积综合征和先天性胆管扩张症患儿作为对照组，留取肝脏组织标本。在光镜和电镜下观察标本的病理改变，以及肝细胞、毛细胆管和肝门成纤维细胞的超微结构。采用SPSS14．0软件，半定量比较BA与对照组肝脏纤维化的差异，检验肝门纤维块成纤维细胞活跃程度与肝纤维化分级的相关性。结果 2005年7月至2006年5月复旦大学附属儿科医院收治的21例BAKasai根治术病例，手术平均年龄（66±20）d；对照组为5例胆汁淤积综合征和10例先天性胆管扩张症患儿。BA组肝组织病理改变主要是肝内门脉区胆管炎症及纤维化形成，肝纤维化程度明显高于同年龄胆汁淤积综合征和先天性胆管扩张症患儿；肝门纤维块毛细胆管增生，部分管腔闭锁、狭窄，腔内炎细胞浸润及部分淤胆，大量间质成分增生；电镜下肝门成纤维细胞活跃、肝脏毛细胆管上皮微绒毛缺失、肝细胞及肝血窦内电子致密物质增多及部分毛细胆管扩张；肝门成纤维细胞分化程度与肝组织纤维化程度相关（P=0．04）。结论 BA肝组织病理改变主要是肝内门脉区胆管炎症及严重纤维化形成；超微结构改变提示肝门部成纤维细胞活跃，其分化程度与肝纤维化程度相关。     

Multiple hepatocellular tumours in a patient treated with oral contraceptives

Summary A case of multiple hepatic tumours in a patient treated for four years with high doses of oral contraceptives is described. Solitary hepatocellular lesions associated with conventional doses of oral contraceptives have been reported previously in twenty nine cases. Haemorrhage has been a common mode of presentation and is attributed to the marked vascularity of the lesions, an appearance referred to as peliosis hepatis. Radiographic studies show this term to be inappropriate as the vessels are of arterial origin. Another finding not previously reported is the presence of diffuse hyperplasia in the non-tumourous parts of the liver.     

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.     

PELIOSIS HEPATIS. An Unusual Case Involving Multiple Organs

The case is that of a 70-year-old Japanese male who had peliosis hepatis with disseminated peliotic lesions in various organs. The initial sign was spontaneous pneumothorax followed by pulmonary hemorrhage. There was no history of anabolic steroid ingestion and his history of pulmonary tuberculosis revealed no pathological changes. Autopsy findings suggested that dilatation of sinusoids with slow progressive injury of sinusoidal endothelium and hepatocytes due to unknown local and general etiologic factors may be an initial mechanism in the development of peliosis hepatis.