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.

A rare case of peliosis hepatis in a patient with chronic renal failure and renal cell carcinoma.

Peliosis hepatis (PH) is a rare disease characterized by the presence of sinusoidal dilation and blood-filled cysts throughout the hepatic parenchyma. We report a case of PH in a 49-year-old woman with chronic renal failure (CRF) on hemodialysis and with renal cell carcinoma (RCC). Dynamic contrast-enhanced computed tomography (CT) showed a 35-mm-diameter, hypervascular tumor in the liver and RCC in the right renal cyst. Ultrasound and superparamagnetic iron oxide-enhanced magnetic resonance imaging were also performed; however, the liver tumor could not be distinguished from the metastasis of RCC. Therefore, echo-guided biopsy of the liver tumor using an 18-G Majima needle was performed. Histological evaluation of the specimen showed irregular sinusoidal dilatation and blood-filled cavities without malignant cells. She was ultimately diagnosed with PH. Subsequently, she underwent total right nephrectomy for RCC and was diagnosed with RCC stage 1 (pT1N0M0). A follow-up CT performed 4 months after nephrectomy showed no growth of PH. Although the development of PH in patients with CRF or RCC who do not undergo renal transplantation is extremely rare, it should be considered in the differential diagnosis to distinguish PH from the metastasis of RCC.

Huge peliosis hepatis mimicking cystic echinococcosis: A case report.

RATIONALE: Peliosis hepatis (PH), which is characterized by blood-filled cavities in the liver, is a rare disease. Its diagnosis depends on postoperative pathological examinations and immunohistochemistry. PATIENT CONCERNS: A 44-year-old female complained of right-middle upper abdominal pain and distension for 1 month, with occasional vomiting and fever. DIAGNOSIS: Because of the similar imaging features, the patient was initially misdiagnosed as cystic echinococcosis (CE). The immunoassay of echinococcosis was negative. Irregular hepatectomy was performed. Eventually, the patient was diagnosed with PH based on postoperative histopathology and immunohistochemistry. INTERVENTIONS: The patient underwent hepatectomy. Then, the cystic lesion was collected for intraoperative pathological examination. Thus, the blood liquid was extracted from the cystic lesion. Pringle maneuver was administered to prevent bleeding, and then the whole cystic lesion was removed. OUTCOMES: She recovered smoothly and there was no relapse occurred during 6 months' follow-up. LESSONS: It is difficult to differentiate PH from CE and other hepatic diseases due to the lack of special imaging features. Pathological examinations and immunohistochemistry can provide a confirmed diagnosis of PH.

Liver Peliosis: A Life-Threatening Condition With No Clear Indication for Liver Transplant.

We present a 21-year-old patient, remarkable for huge hepatomegaly with the liver, occupying almost the entire abdominal cavity, and mild portal hypertension due to splenic vein compression. After ultrasonography-guided liver biopsy, performed to establish the diagnosis, the patient had bleeding from the liver. Fortunately, emergency laparotomy was started immediately, and the patient was saved. Macroscopically, the liver appeared to be of purple-red color, flabby to the touch, and able to be easily wrinkled with fingers. When all available clinical data were considered, a diagnosis of liver peliosis was made. The patient was recommended close follow-up at the specialized liver surgery clinic with access to emergency surgical procedures, including liver transplant.

Peliosis hepatis: 2 case reports of a rare liver disorder and its differential diagnosis.

RATIONALE: Peliosis hepatis (PH) is a rare tumor-like liver lesion composed of multiple blood-filled cavities within the liver parenchyma. It is hard to differentiate PH from other liver lesions by imaging, such as carcinoma, metastases, or abscess. PATIENT CONCERNS: Here, we reported 2 cases that presented with liver lesions under ultrasound and computed tomography (CT) scanning, without any history of liver diseases or drug usage traced back. DIAGNOSES: Liver biopsy and laparoscopy were processed, and the lesions were eventually diagnosed as PH by histopathology, which microscopically presented with multiple sinusoidal dilatations with blood-filled cystic spaces. INTERVENTIONS: After the liver biopsy or laparoscopy, the patients were discharged and followed up in the clinic. OUTCOMES: Both patients were followed up for at least 1 year with good recovery. LESSONS: PH should always be recognized in the differentiation of liver lesions, particularly indistinctive lesion(s) without any history of liver-related diseases.

Contrast enhanced ultrasound of a rare case of Peliosis hepatis.

Peliosis hepatis is a rare benign disorder of the liver, histologically characterized by blood-filled cystic cavities of various sizes and irregular shapes, communicating with the hepatic sinusoids. Only a few cases of peliosis hepatis have been described using contrast enhanced ultrasound showing admittedly various dynamic enhancement patterns. We present a case of peliosis hepatis with a typical target-sign enhancement depicted by means of contrast enhanced ultrasound.

Peliosis Hepatis Mimicking Malignant Hypervascular Tumors.

BACKGROUND: Peliosis hepatis is an uncommon vascular condition characterized by multiple, randomly distributed, blood-filled, and cyst-like cavities throughout the liver. The unique clinical nature consisting of an asymptomatic, benign, and incidental finding sometimes conceals itself even until autopsy. However, the exact pathomechanism remains as yet unproven. METHOD: We encountered the patient with multiple hypervascular masses in the whole liver and investigated the clinicopathological features underlying this condition. RESULTS: Detailed herein is a case of peliosis hepatis on the basis of our findings. Our patient illustrated a lethal instance which mimicked a malignancy although it is usually an indolent finding. CONCLUSION: Peliosis hepatis should always be borne in mind as a differential diagnosis of atypical hypervascular hepatic lesion, especially in patients with clinical conditions described in the text.

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.

[Focal hepatic peliosis without evident aetiology].

Hepatic peliosis is a rare vascular disorder of the liver characterized by small cavities containing blood. It is most often asymptomatic but may give rise to portal hypertension and even fatal liver rupture. In this case a 47-year-old woman presented with abdominal pain and elevated alkaline phosphatase. An ultrasonography and additional MRI of the liver detected three lesions suspected of focal nodular hyperplasia. A core needle biopsy revealed that hepatic peliosis was the correct diagnosis. No causative factors were found and the patient was followed by consecutive MRIs.

Peliosis hepatis in a kidney transplant recipient with manifestation as massive ascites and liver dysfunction: case report.

We report a case of 59-year-old woman who received a kidney transplant 7 years earlier without evidence of viral hepatitis history. She was asymptomatic initially and a newly developed nodule, ∼2.3 cm in size, was discovered in the right liver during routine sonographic examination. Computerized tomography-guided biopsy was inconclusive at that time. However, the lesion grew to 6.8 cm and bilobular multiple nodules developed with concomitant massive ascites and hyperbilirubinemia months later. Laparoscopy showed typical bluish-reddish-blackish nodules. Needle-biopsy histology showed severe sinusoid dilation and dropout of centrilobular hepatocytes consistent with peliosis hepatis. Reticulin staining also demonstrated disruption of sinusoidal reticulin fibers. We tried to withdraw possible offending drugs to anticipate regression of peliosis, but it failed and liver dysfunction progressed, leaving liver transplant as the last resort in such rare circumstances.

Peliosis hepatis due to disseminated tuberculosis in a patient with AIDS.

Peliosis hepatis is a rare histopathological entity of unknown etiology. We present a case of peliosis hepatis in a 44-year-old man with disseminated tuberculosis and acquired immunodeficiency syndrome. The diagnosis of peliosis hepatis was based on liver biopsy results which were suggestive of tuberculous etiology. Diagnosis of tuberculosis was confirmed by auramine stain, rRNA amplification and culture of Mycobacterium tuberculosis from synovial fluid of the elbow joint. The patient responded favourably to tuberculostatic treatment with four drugs and the early initiation of highly active antiretroviral therapy. Histopathological evidence of peliosis hepatis, without an obvious cause, makes it necessary to rule out tuberculosis, especially in the context of immunodeficiency diseases and immigrants from endemic areas.

[Primary hepatic angiosarcoma: a clinical and pathological analysis].

OBJECTIVE: To investigate the clinicopathological characteristics, differential diagnosis, and prognosis of primary hepatic angiosarcoma, and to review the literature. METHODS: Twenty cases of primary hepatic angiosarcoma were analyzed by gross examination and light microscopy. Immunostaining was performed to detect the expression of CD34, CD31, FVIIIRAg, CK, GPC-3, Hepatocyte,vimentin, PTEN, desmin, and CD117. RESULTS: The age of the patients ranged from 7 to 86 years. Eleven cases were male, and 9 were female. All cases showed no specific clinical manifestations and imaging results. Macroscopically, the tumors showed diffuse multi-nodular or single nodular patterns with hemorrhage. Microscopically, there were various patterns such as cavernous vascular space and epithelioid hemangioendothelioma-like appearances; however, specific pathological diagnostic features of angiosarcoma still existed in all cases. All of the cases expressed at least one of the three immunohistochemical markers: CD31, CD34 and/or FVIIIRag. Ten cases had PTEN low expression. Ki-67 proliferative index was more than 10% in all cases. None of cases expressed desmin, CD117, GPC-3 or Hepatocyte. CONCLUSIONS: Primary hepatic angiosarcoma is a rare malignant tumor. Detailed morphological observation and using various vascular endothelial immunohistochemical markers can help to establish the diagnosis accurately.

Fatal hepatic hemorrhage by peliosis hepatis in X-linked myotubular myopathy: a case report.

We report a 5-year-old boy with X-linked myotubular myopathy complicated by peliosis hepatis. At birth, he was affected with marked generalized muscle hypotonia and weakness, which required permanent ventilatory support, and was bedridden for life. He died of acute fatal hepatic hemorrhage after using a mechanical in-exsufflator. Peliosis hepatis, defined as multiple, variable-sized, cystic blood-filled spaces through the liver parenchyma, was confirmed by autopsy. To avoid fatal hepatic hemorrhage by peliosis hepatis, routine hepatic function tests and abdominal imaging tests should be performed for patients with X-linked myotubular myopathy, especially at the time of using artificial respiration.