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 in a Child with X-Linked Myotubular Myopathy Treated with Living-Donor Liver Transplant: A Case Report.

BACKGROUND: Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. CASE REPORT: We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis. CONCLUSIONS: Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

An autopsy case of peliosis hepatis with X-linked myotubular myopathy.

This report describes the autopsy case of a 4-year-old boy who died from hepatic hemorrhage and rupture caused by peliosis hepatis with X-linked myotubular myopathy. Peliosis hepatis is characterized by multiple blood-filled cavities of various sizes in the liver, which occurs in chronic wasting disease or with the use of specific drugs. X-linked myotubular myopathy is one of the most serious types of congenital myopathies, in which an affected male infant typically presents with severe hypotonia and respiratory distress immediately after birth. Although each disorder is rare, 12 cases of pediatric peliosis hepatis associated with X-linked myotubular myopathy have been reported, including our case. Peliosis hepatis should be considered as a cause of hepatic hemorrhage despite its low incidence, and it requires adequate gross and histological investigation for correct diagnosis.

Spontaneous hepatic rupture in a bodybuilder: a case report and review of the literatura

This article is the first description of a spontaneous hepatic rupture in a young bodybuilder with a history of clenbuterol and ephedrine alkaloid use. The patient presented with a sudden mid-epigastric pain and vomiting. Hemoglobin levels decreased a few hours later and a computed tomography scan was performed which revealed a rupture of the right liver capsule and hemoperitoneum. Two attempts at transarterial embolization did not control the bleeding and a right hemihepatectomy was performed. The pathological report identified a hepatic adenoma, a capsular tear and diffuse peliosis hepatis. The patient was discharged in a good condition after eleven days. Spontaneous hepatic ruptures are rare and life-threatening and are usually described in association with tumors, connective tissue diseases and gestosis. This article is a review of the available literature with regard to this condition, with a focus on its relation to peliosis hepatis and banned substance used by body image fanatics. The present case highlights the challenging diagnosis of this potentially fatal liver complication in a healthy appearing male, the risk associated with the online trade of performance enhancing drugs and its relation with peliosis hepatis

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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.

Spontaneous hepatic rupture in a bodybuilder: a case report and review of the literature.

This article is the first description of a spontaneous hepatic rupture in a young bodybuilder with a history of clenbuterol and ephedrine alkaloid use. The patient presented with a sudden mid-epigastric pain and vomiting. Hemoglobin levels decreased a few hours later and a computed tomography scan was performed which revealed a rupture of the right liver capsule and hemoperitoneum. Two attempts at transarterial embolization did not control the bleeding and a right hemihepatectomy was performed. The pathological report identified a hepatic adenoma, a capsular tear and diffuse peliosis hepatis. The patient was discharged in a good condition after eleven days. Spontaneous hepatic ruptures are rare and life-threatening and are usually described in association with tumors, connective tissue diseases and gestosis. This article is a review of the available literature with regard to this condition, with a focus on its relation to peliosis hepatis and banned substance used by body image fanatics. The present case highlights the challenging diagnosis of this potentially fatal liver complication in a healthy appearing male, the risk associated with the online trade of performance enhancing drugs and its relation with peliosis hepatis.

Peliosis hepatis: Personal experience and literature review.

Peliosis hepatis (PH) is a disease characterized by multiple and small, blood-filled cysts within the parenchymatous organs. PH is a very rare disease, more common in adults, and when it affects the liver, it comes to the surgeon's attention only in an extremely urgent situation after the lesion's rupture with the resulting hemoperitoneum. This report describes the case of a 29-year-old woman affected by recurring abdominal pain. Computed tomography scans showed a hepatic lesion formed by multiple hypodense areas, which showed an early acquisition of the contrast during the arterial phase. Furthermore, it remained isodense with the remaining parenchyma during the late venous phase. We decided on performing a liver resection of segment VII while avoiding a biopsy for safety reasons. The histopathologic examination confirmed the diagnosis of focal PH. PH should always be considered in the differential diagnosis of hepatic lesions. Clinicians should discuss the possible causes and issues related to the differential diagnosis in addition to the appropriate therapeutic approach. The fortuitous finding of a lesion, potentially compatible with PH, requires elective surgery with diagnostic and therapeutic intents. The main aim is to prevent the risk of a sudden bleeding that, in absence of properly equipped structures, may have a fatal outcome.

Peliosis hepatis disseminated rapidly throughout the liver in a patient with prostate cancer: a case report.

INTRODUCTION: In the World Health Organization histological classification of the liver tumor, peliosis hepatis is defined as a tumor-like lesion. The entity is characterized by the appearance of multiple cyst-like, blood-filled spaces within the liver parenchyma. CASE PRESENTATION: A 77-year-old Japanese man with prostate cancer was referred to our department because he was diagnosed as having two hepatic tumors. The tumors were confirmed to be peliosis hepatis by repeated needle biopsies and because of their atypical images by enhanced computed tomography and enhanced magnetic resonance imaging. Later these tumors grew rapidly, increased in number, and disseminated throughout his whole liver. We are now treating the patient conservatively due to his age and his existing medical conditions. CONCLUSION: Peliosis hepatis is a rare hepatic benign tumor that should be considered in the differential diagnosis of multiple unknown liver tumors that are revealed by atypical radiological images.

Spontaneous hepatic rupture during pregnancy in a patient with peliosis hepatis.

Spontaneous hepatic rupture (SHR) during pregnancy is a rare but well known complication and it usually occurs alongside eclampsia or HELLP syndrome. SHR in uncomplicated pregnancy is extremely rare and can be associated to different undiagnosed pathological conditions. We report the case of a nulliparous woman, 27 weeks pregnant, with a peliosis hepatis, previously unknown, who was admitted to our unit due to SHR and massive hemoperitoneum. The conception was obtained by embryo transfer after multiple attempts of hormone-supported cycles using estrogens and progesterone. After emergency laparotomy the patient was submitted to deliver of the dead foetus and damage control of the hepatic bleeding source. At relaparotomy a right posterior sectionectomy (segments VI and VII) and segmentectomy of segment V were performed. The patient was discharged in good physical conditions after 18 days from admission. If hepatic rupture is suspected in a pregnant patient a collaborative multidisciplinary approach is mandatory. The cornerstones of medical and surgical management are highlighted. At the best of our knowledge this is the first case of SHR in a pregnant woman with peliosis hepatis. A possible correlation of an increased risk for SHR in a pregnant patient who was submitted to several attempts for embryo transfer is discussed. The relevant scientific literature of the possible causative role of the estrogen therapy in inducing politic liver damage is also reviewed.

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 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.

Rotura hepática secundaria a peliosis hepática / Ruptured liver caused by peliosis hepatis

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Peliosis hepatis: a complicating finding in a case of biliary colic.

A 33-year-old woman was referred for an ultrasound of the abdomen because of biliary colic. The symptoms had started 2 months after giving birth to her first child. The ultrasound showed gallstones, but it also revealed multiple focal liver lesions that were initially thought to be malignant. The examination was supplemented with a CT scan, contrast-enhanced ultrasound (CEUS) and MRI. The lesions were suspected to be peliosis hepatis-a rare morphological entity characterised by multiple blood-filled cavities in the liver. Because of uncertainty as to the aetiology of the lesions demonstrated at CEUS and MRI, the diagnosis was definitively confirmed by large-size needle biopsies. Regular size biopsies were initially insufficient for diagnosis. The use of oral contraceptives for several years or the recent pregnancy may have been the cause of peliosis hepatis in this patient.

Peliosis hepatis presenting as liver rupture in a vulnerable adult: a case report.

Liver rupture is a serious, life-threatening event that is commonly due to blunt abdominal trauma, which should be suspected in a patient who is unconscious or unable to communicate. We report an autopsy case of a 28-year-old woman with severe developmental delay who presented to the emergency department with hemoperitoneum due to massive liver rupture and subsequently died without a diagnosis. An autopsy performed by the hospital pathology department confirmed hemoperitoneum due to hepatic rupture. The case was then referred to the medical examiner to exclude a traumatic etiology. After review of the clinical data, radiological images, and gross and microscopic pathological features, a diagnosis of peliosis hepatis was established. This rare entity has been reported previously as a cause of spontaneous, nontraumatic liver rupture and is reported here to demonstrate its characteristic features and potential to present as fatal hepatic rupture in circumstances in which occult injury must be excluded.