Haemophagocytic lymphohistiocytosis associated with bartonella peliosis hepatis following kidney transplantation in a patient with HIV.

Bacillary peliosis hepatis is a well recognised manifestation of disseminated Bartonella henselae infection that can occur in immunocompromised individuals. Haemophagocytic lymphohistiocytosis is an immune-mediated condition with features that can overlap with a severe primary infection such as disseminated Bartonella spp infection. We report a case of bacillary peliosis hepatis and secondary haemophagocytic lymphohistiocytosis due to disseminated Bartonella spp infection in a kidney-transplant recipient with well controlled HIV. The patient reported 2 weeks of fever and abdominal pain and was found to have hepatomegaly. He recalled exposure to a sick dog but reported no cat exposures. Laboratory evaluation was notable for pancytopenia and cholestatic injury. The patient met more than five of eight clinical criteria for haemophagocytic lymphohistiocytosis. Pathology review of a bone marrow core biopsy identified haemophagocytosis. A transjugular liver biopsy was done, and histopathology review identified peliosis hepatis. Warthin-Starry staining of the bone marrow showed pleiomorphic coccobacillary organisms. The B henselae IgG titre was 1:512, and Bartonella-specific DNA targets were detected by peripheral blood PCR. Treatment with doxycycline, increased prednisone, and pausing the mycophenolate component of his transplant immunosuppression regimen resulted in an excellent clinical response. Secondary haemophagocytic lymphohistiocytosis can be difficult to distinguish from severe systemic infection. A high index of suspicion can support the diagnosis of systemic Bartonella spp infection in those who present with haemophagocytic lymphohistiocytosis, especially in patients with hepatomegaly, immunosuppression, and germane animal exposures.

Peliosis Hepatis with Chylous Ascites in a Dog.

A 6-year-old spayed female Toy Poodle dog was referred to the Hokkaido University Veterinary Teaching Hospital for abdominal distension. Abdominocentesis yielded ascitic fluid that had a mildly increased total protein concentration and a 2.7-fold higher triglyceride concentration than plasma, and was interpreted as chylous ascites. The patient had an enlarged liver, which contained multiple, small, nodular masses and cyst-like structures. Microscopically, these lesions were multifocal dilated spaces containing lymphocytes, endothelial cells, fibrin and islands of hepatocytes. Increased α-smooth muscle actin-positive cells were observed in hepatic sinusoids. Based on these findings, we diagnosed peliosis hepatis with chylous ascites, which is likely to have been due to lymphangiectasia and disrupted hepatic sinusoids. Neither Bartonella spp DNA nor mutations in ACVRL1 and MTM1 genes were detected, although there was a 47-fold increase in hepatic ACVRL1 expression compared with age-matched control liver. To the authors' knowledge, this is the first report of chylous ascites resulting from peliosis hepatis in any species.

Resolution of spontaneous hemoabdomen secondary to peliosis hepatis following surgery and azithromycin treatment in a Bartonella species infected dog.

OBJECTIVE: To describe a case of hemoperitonium in a dog with Bartonellosis and peliosis hepatis (PH) lesions that resolved following antimicrobial therapy. CASE SUMMARY: A 3-year-11-month-old 22.5 kg female spayed mixed breed dog presented for progressive lethargy and vomiting. An abdominal ultrasonographic examination revealed moderate ascites, which when sampled was nonclotting hemorrhagic fluid. An exploratory laparotomy revealed a large volume of nonclotted blood in the dog's abdomen and blood-filled vesicular lesions dispersed diffusely along multiple lobes of the liver. Biopsies revealed lesions indicative of PH. Serology testing for Bartonella species was positive. Treatment with azithromycin resulted in Bartonella serology negative status and no further evidence of hemoperitonium at recheck examination 12 months after initial presentation. NEW OR UNIQUE INFORMATION PROVIDED: This is the first reported case of PH and hemoperitoneum in a Bartonella species serology positive dog wherein treatment with azithromycin resulted in serology negative status. There have been no subsequent episodes of hemoperitoneum in the 12 months since treatment.

Peliosis hepatis in cats is not associated with Bartonella henselae infections.

Peliosis hepatis is a vasculoproliferative disorder of the liver with infectious and noninfectious causes. In humans and dogs, Bartonella henselae has been linked to peliosis hepatis. Although domestic cats are the natural reservoir of B. henselae and although peliosis hepatis is common in this species, an association between this condition and infection with B. henselae has never been investigated in cats. In this study, 26 cases of peliosis hepatis in cats were tested for B. henselae infection by nested polymerase chain reaction and immunohistochemistry. The authors failed to detect B. henselae nucleic acid or antigen in any of the affected liver specimens. These findings suggest that, unlike in humans and dogs, peliosis hepatis in cats may not be significantly associated with a B. henselae infection.

Pathology of captive moustached tamarins (Saguinus mystax).

The pathology of 33 moustached tamarins (Saguinus mystax) previously used in hepatitis A and GB virus studies is reported. Chronic lesions in colon, heart, and kidney were common in the monkeys and appeared not to be due to the experimental exposures. Colitis cystica profunda (CCP), a disease that affects humans and is characterized by the presence of mucin-filled epithelial downgrowths and cysts in the colonic submucosa, was found in 24 of the 33 (72.7%) tamarins. Interstitial myocardial fibrosis was present in 22 (66.6%) animals, and various degrees of membranoproliferative glomerulonephritis occurred in 28 (84.8%) monkeys. In addition, 28 (84.8%) tamarins demonstrated diffuse hepatocellular vacuolation with mild lymphoplasmacytic infiltrates, possibly as a result of the experimental infections, and peliosis hepatis occurred in 7 (21.2%) animals. The etiology of CCP is unknown, and no reliable animal models are available because most cases in animals are reported only sporadically. Myocardial fibrosis in tamarins has not been reported previously, and all current animal models require experimental manipulation of the animal to mimic the human disease. The results from this study suggest that captive S. mystax has high incidence of spontaneous CCP, myocardial fibrosis, and membranoproliferative glomerulonephritis. This species may be a spontaneous animal model for pathogenesis and experimental therapy studies of the analogous human diseases.

Peliosis hepatis-like lesion in a pekingese dog. A case report.

Peliosis hepatis (PH) is a rare condition characterized by dilatation of hepatic sinusoids and the presence of blood-filled spaces within the liver. The disease occurs mainly in subjects exposed to toxic substances or estrogens, and is often asymptomatic. A 9 year-old pekingese female dog was sent to Division of Pathology for autopsy because of sudden death of unclear cause. Autopsy revealed severe hydroperitoneum (1500 ml of transsudate), dilatation of heart ventricles, passive hyperemia of lungs, spleen and kidneys. The liver was enlarged, with slightly wrinkled surface, with many red spots, of different size and shape. Microscopic examination of the liver revealed the presence of areas with severe dilatation of hepatic sinusoids, and multiple cystic spaces filled with blood. Some of these spaces were lined with endothelium and other were not. Spaces were different in shape and size and were localized in the areas between dilated sinusoids. Based on the macroscopic and microscopic examination, the liver lesions were identified as severe hepatic sinusoidal dilatation with formation of peliosis hepatis-like structures.

Peliod hepatocellular carcinoma in a domesticated ferret (Mustela putorius furo).

Peliod hepatocellular carcinoma was diagnosed in a domesticated ferret (Mustela putorius furo). The diagnosis was made using immunohistochemical analysis, histologic examination, and the accepted classification schemes based on histomorphologic features. Bilateral, adrenocortical hyperplasia also was evident. Speculation about a possible association between the variant of hepatocellular neoplasia diagnosed in this animal and its adrenal pathologic changes was done.

Histological evidence of disease in wild stoats (Mustela erminea) in England.

Forty-four free-living stoats were collected from five game estates in England and examined for evidence of disease. All the macroscopic lesions were attributable to the trauma associated with being trapped or shot. There were no significant microscopic lesions in 27 (61 per cent) of the stoats. There was evidence of nematode parasitism in the intestines of six stoats (14 per cent), and in the lungs of five stoats (11 per cent), and of pulmonary granulomatous inflammation or microgranulomas in five stoats. In two of the stoats, blood-filled cavities in the liver were suggestive of peliosis hepatis, possibly associated with infection by Bartonella species or with sublethal exposure to anticoagulant rodenticides.

Peliosis hepatis in a dog infected with Bartonella henselae.

A 6-year-old spayed female Golden Retriever was examined because of generalized weakness and abdominal distention. Abdominal ultrasonography revealed a large quantity of peritoneal fluid. In addition, the liver appeared larger than normal and contained multiple, small, nodular masses and cyst-like structures. Abdominal exploratory surgery was performed, and 5 L of serosanguineous peritoneal fluid was removed. Gross lesions were not found in the stomach, kidneys, intestines, adrenal glands, or urinary bladder. There were diffuse cystic nodules in all liver lobes. The dog did not recover from anesthesia. A diagnosis of peliosis hepatis was made on the basis of gross and histologic appearance of the liver. A polymerase chain reaction assay revealed Bartonella henselae DNA in liver specimens. To our knowledge, this is the first report of molecular evidence of B henselae infection in a dog with peliosis hepatis.

Phlebectatic peliosis hepatis in Australian cattle.

Peliosis hepatis can be produced predictably in cattle following regular oral dosing for several weeks with dried plant of various Pimelea spp which contain irritant diterpenoid daphnane esters such as simplexin . The condition is associated with a massive hypervolemia and cor pulmonale. Affected animals may recover after removal of the toxin from the diet. The mechanism of peliosis hepatis in man and other species is obscure, and the present model in the large domestic species could well be employed in investigations of the pathogenesis of this lesion. The condition appears to have its origin as a portal venular ectasia and can be regarded as a true phlebectatic peliosis hepatis. Blood volume measurements may be of value in forecasting the possible development of peliosis hepatis in humans, which may occur in association with regular long-term anabolic steroid therapy.

Peliosis hepatis-like lesion in aging rats.

The incidence of peliosis hepatis-like lesions in two-year-old Charles River-CD Sprague-Dawley rats (Charles River Breeding Laboratories, Wilmington, MA) was almost twice as high in males as females. Blood lakes developed from progressive expansion of sinusoids with destruction of the hepatic cell cords. The endothelial processes of the blood lakes had edematous swelling, vesiculation, and denudation. The microvilli of hepatocytes became swollen, blunted, and sloughed where the endothelial processes were disrupted. Subsequently, the hepatocytes were exposed directly to circulating blood cells and had degenerative changes with accumulation of red blood cells in the cytoplasm. The membrane-bound cysts were observed with the blood lakes and were bounded with atrophic hepatocytes or membranous wall extending to the Disse's space of adjacent hepatocytes. The cysts were filled with proteinaceous fluid, fibrin, and a few red blood cells. The wall of membrane-bound cysts consisted of endothelial processes extending from the endothelial cells of adjacent hepatocytes. The wall appeared to develop from adhesion of endothelial processes surrounding sinusoidal spaces of the hepatocytes following lysis of atrophic hepatocytes.