Peliosis hepatis complicated by portal hypertension following renal transplantation.

Peliosis hepatis is a rare benign disease, but in last years the number of identified cases has increased. This disease is known to be sometimes accompanied by hepatocellular carcinoma. In the recent article, Yu et al describe a case of liver peliosis, characterized by an increased proliferative index. Therefore, additional diagnosis of patients should include analyzing other tumor markers expression in order to assess the risk of malignant cell transformation in peliosis hepatis.

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.

Peliosis hepatis and systemic lupus erythematosus: A rare condition identified by magnetic resonance imaging.

Peliosis hepatis is a rare benign disorder characterized by the presence of multiple cavities filled with blood with no preferential localization in the liver parenchyma. It may be related to several etiologic conditions, especially infections and toxicity of immunosuppressive drugs. To our knowledge, there are only three articles reporting the association between peliosis hepatis and systemic lupus erythematosus. In this report, we describe a case of this rare condition, highlighting the importance of magnetic resonance imaging. A short review of this subject is also presented.

Peliosis hepatis and systemic lupus erythematosus: A rare condition identified by magnetic resonance imaging / Peliose hepática e lúpus eritematoso sistêmico: uma rara condição identificada pela ressonância nuclear magnética

Summary Peliosis hepatis is a rare benign disorder characterized by the presence of multiple cavities filled with blood with no preferential localization in the liver parenchyma. It may be related to several etiologic conditions, especially infections and toxicity of immunosuppressive drugs. To our knowledge, there are only three articles reporting the association between peliosis hepatis and systemic lupus erythematosus. In this report, we describe a case of this rare condition, highlighting the importance of magnetic resonance imaging. A short review of this subject is also presented.

Resumo Peliose hepática é uma patologia benigna rara caracterizada pela presença de múltiplas cavidades preenchidas por sangue sem localização preferencial no parênquima do fígado. Pode estar relacionada a uma série de condições etiológicas, dentre elas doenças infecciosas e toxicidade por drogas imunossupressoras. Para nosso conhecimento, existem apenas três artigos que abordam a associação entre peliose hepática e lúpus eritematoso sistêmico. Neste relato, descrevemos um caso desta rara condição, destacando a importância da ressonância magnética. Uma breve revisão sobre o tema é apresentada.

Peliosis hepatis complicated by portal hypertension following renal transplantation.

Peliosis hepatis (PH) is a vascular lesion of the liver that mimics a hepatic tumor. PH is often associated with underlying conditions, such as chronic infection and tumor malignancies, or with the use of anabolic steroids, immunosuppressive drugs, and oral contraceptives. Most patients with PH are asymptomatic, but some present with abdominal distension and pain. In some cases, PH may induce intraperitoneal hemorrhage and portal hypertension. This study analyzed a 46-year-old male who received a transplanted kidney nine years prior and had undergone long-term immunosuppressive therapy following the renal transplantation. The patient experienced progressive abdominal distention and pain in the six months prior to this study. Initially, imaging studies revealed multiple liver tumor-like abnormalities, which were determined to be PH by pathological analysis. Because the hepatic lesions were progressively enlarged, the patient suffered from complications related to portal hypertension, such as intense ascites and esophageal varices bleeding. Although the patient was scheduled to undergo liver transplantation, he suffered hepatic failure and died prior to availability of a donor organ.

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.

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.

Peliosis hepatis in a child with myotubular myopathy: successful treatment using hepatic artery embolization.

Peliosis hepatis (PH) is a rare condition characterized by multiple blood-filled spaces within the hepatic parenchyma that can lead to fatal hemorrhage. There is no consensus on the best treatment algorithm for PH, and therapy is directed at removing the potential causative agent with operative intervention when necessary. Here we present the first known case of PH in a child with myotubular myopathy who was successfully treated with angiography and hepatic artery embolization as a first line therapy, without the need for operative intervention. Awareness of this condition and the available treatment modalities may lead to favorable outcomes in future cases.

Surgical treatment of a patient with peliosis hepatis: a case report.

This report describes a case of a space-occupying lesion in the right liver in a 38-year-old man who was found to have peliosis hepatis. Clinical data of this patient were presented, including medical history, laboratory test and imaging results, and postoperative pathological findings (hematoxylin and eosin staining). Review of his medical history showed that the patient had been bitten by a dog three years earlier. B-mode ultrasonography revealed an uneven echo mass in the right hemiliver, and magnetic resonance imaging scans also showed a mass in the anterior segment of the right liver. Upon surgical removal, the mass was found to be 4.0 cm × 3.8 cm × 3.8 cm in size and located in segment VI. The mass had a dark and soft appearance, with an irregular edge on intraoperative ultrasonography. Postoperative pathological findings revealed many small capsules filled with blood cells. The patient was diagnosed with peliosis hepatis based on his medical history of having been bitten by a dog, presence of mild anemia, and lack of characteristic symptoms, including fever of unknown origin, abdominal pain, and hepatosplenomegaly, combined with intraoperative and postoperative pathologic findings. The operation was successful, and after being treated with anti-infection agents, the patient had a good recovery.

Adhesins of Bartonella spp.

Adhesion to host cells represents the first step in the infection process and one of the decisive features in the pathogenicity of Bartonella spp. B. henselae and B. quintana are considered to be the most important human pathogenic species, responsible for cat scratch disease, bacillary angiomatosis, trench fever and other diseases. The ability to cause vasculoproliferative disorders and intraerythrocytic bacteraemia are unique features of the genus Bartonella. Consequently, the interaction with endothelial cells and erythrocytes is a focus in Bartonella research. The genus harbours a variety of trimeric autotransporter adhesins (TAAs) such as the Bartonella adhesin A (BadA) of B. henselae and the variably expressed outer-membrane proteins (Vomps) of B. quintana, which display remarkable variations in length and modular construction. These adhesins mediate many of the biologically-important properties of Bartonella spp. such as adherence to endothelial cells and extracellular matrix proteins and induction of angiogenic gene programming. There is also significant evidence that the laterally acquired Trw-conjugation systems of Bartonella spp. mediate host-specific adherence to erythrocytes. Other potential adhesins are the filamentous haemagglutinins and several outer membrane proteins. The exact molecular functions of these adhesins and their interplay with other pathogenicity factors (e.g., the VirB/D4 type 4 secretion system) need to be analysed in detail to understand how these pathogens adapt to their mammalian hosts.

Anabolic androgenic steroids abuse and liver toxicity.

In the athletes the wide use of Anabolic Androgenic Steroids (AAS) cause series damage in various organs, in particular, analyzing the liver, elevation on the levels of liver enzymes, cholestatic jaundice, liver tumors, both benign and malignant, and peliosis hepatis are described. A prolonged AAS administration provokes an increase in the activities of liver lysosomal hydrolases and a decrease in some components of the microsomal drug-metabolizing system and in the activity of the mitochondrial respiratory chain complexes without modifying classical serum indicators of hepatic function. Liver is a key organ actively involved in numerous metabolic and detoxifying functions. As a consequence, it is continuously exposed to high levels of endogenous and exogenous oxidants that are by-products of many biochemical pathways and, in fact, it has been demonstrated that intracellular oxidant production is more active in liver than in tissues, like the increase of inflammatory cytokines, apoptosis and the inhibitors of apoptosis NF- κB and Heat Shock Proteins.

[Bartonella henselae, an ubiquitous agent of proteiform zoonotic disease]. / Bartonella henselae, un agent d'infections ubiquitaires.

Bartonella henselae is the causative agent of cat scratch disease, a human infection usually characterized by persistent regional lymphadenopathy. It is transmitted to humans by cat scratches or bites. Cats are the major reservoir for this bacterium thus B. henselae has a worldwide distribution. The bacterial pathogenicity may bay emphasized by the immune status of the infected host. Angiomatosis or hepatic peliosis are the most frequent clinical manifestations in immunocompromised patients. B. henselae is also responsible for endocarditis in patients with valvular diseases, and may induce various clinical presentations such as: bacteriemia, retinitis, musculoskeletal disorders, hepatic or splenic diseases, encephalitis, or myocarditis. Several diagnostic tools are available; they may be combined and adapted to every clinical setting. B. henselae is a fastidious bacterium; its diagnosis is mainly made by PCR and blood tests. No treatment is required for the benign form of cat scratch disease. For more severe clinical presentations, the treatment must be adapted to every clinical presentation.

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.

Peliosis hepatis como complicación del uso de anticonceptivos orales en una paciente con mielodisplasia / Peliosis hepatis as a complication of the ise of oral contraceptives in a patient with myelodysplasia

Introduction: Peliosis hepatis (PH) is an uncommon condition in pediatrics; however, it is one of the most serious complications associated with the long-term use of use of steroids. It is characterized by multiple blood-filled cavities, mostly involving the liver. Myelodysplastic Syndrome (MDS) is also a complex and infrequent hematological condition; it may transform into acute leukemia and its treatment requires medications that may lead to PH. Case Report: 13 year-old girl with MDS, refractory cytopenia type. A family donor for SCL was not available, therefore immunosuppressive treatment, steroids and transfusions were initiated. Due to metrorrhage, estrogen was used at high doses. She developed acute abdominal pain; abdominal ultrasound and CL scan showed PH and peritoneal bleeding. Oral contraceptives were decreased resulting in reduction of PH, but a new episode of uterine bleeding causing hypovolemic shock forced a hysterectomy in order to suspend estrogen treatment. Due to lack of response to treatment to SMD, she continued been treated with transfusions as needed, and died 32 months post diagnosis. Discussion: PH is an uncommon and life-threatening condition in children receiving prolonged treatment with steroids. Current modalities of SCL in patients with MDS will replace the need for steroids, thus avoiding this severe complication.

Introducción: La Peliosis Hepática (PH) es una condición muy infrecuente en pediatría, caracterizada por la presencia de múltiples cavidades sanguíneas en el parénquima hepático, asociada al uso prolongado de estrógenos o corticoides, El Síndrome Mielodisplásico (SMD) es una alteración hematológica compleja que puede evolucionar a leucemia y que puede requerir para su tratamiento medicamentos relacionados al desarrollo de PH. Caso Clínico: Niña 13 años, con SMD tipo citopenia refractaria, con dependencia transfusional, sin posibilidad de realizar Trasplante de progenitores hematopoyéticos (TPH) por falta de donante familiar compatible. Recibió transfusiones, inmunosupresores y corticoides por tiempo prolongado. Presentó metrorragias severas requiriendo estrógenos en altas dosis. Evolucionó con hemoperitoneo, diagnosticándose PH por ecografía y scanner abdominal. Al reducir dosis de estrógenos disminuyeron lesiones hepáticas, pero nuevo episodio de metrorragia con shock hipovolémico, obligó a realizar histerectomía para suspender estrógenos. Sin respuesta a tratamiento del SMD, se mantuvo con transfusiones según requerimiento y falleció a los 32 meses del diagnóstico. Discusión: La PH es una complicación grave, que podría evitarse con el desarrollo de nuevas técnicas de TPH que permiten contar con donantes no relacionados para el tratamiento de síndromes de falla medular como el SMD.

Peliosis hepatis following treatment with androgen-steroids in patients with bone marrow failure syndromes.

Androgens widely used in the treatment of bone marrow failure syndromes can in rare cases cause hepatic peliosis, a pathological entity characterized by multiple blood-filled cavities in the liver parenchyma. Bone marrow failure syndromes per se are associated with a low coagulation status, which is further magnified by bone marrow transplantation for aplastic anaemia due to deep thrombocytopenia. Both these conditions can cause bleeding; their combination is especially dangerous. We describe two cases of aplastic anaemia due to paroxysmal nocturnal hemoglobinuria and Fanconi syndrome, in which patients developed peliosis hepatis after prolonged treatment with androgens. One patient developed severe subcapsular bleeding, successfully treated with catheterization of the right hepatic artery and embolization of the bleeding site. The second patient bridged over deep post-transplant aplasia with high frequency platelet transfusions, and demonstrated an uncomplicated post-BMT course. We suggest avoiding or interrupting treatment with androgens in patients preparing for BMT.

Dopaminergic D2 receptor knockout mouse: an animal model of prolactinoma.

Dopamine receptor type 2 (D2R) knockout mice (KO) have chronic hyperprolactinemia, pituitary hyperplasia, and a moderate decrease in MSH content. They are also growth retarded evidencing an alteration in the GH-IGF-I axis. In D2R KO, lactotropes do not show dense secretory granules but degranulated cells and fewer somatotropes, gonadotropes and thyrotropes. Prolactin levels are always higher in female than in male knockouts, and in accordance, pituitary hyperplasia is observed at 8 months only in females. After 16 months of age, highly vascularized adenomas develop, especially in females. Prominent vascular channels in the hyperplastic and adenomatous pituitaries, as well as extravasated red blood cells not contained in capillaries is also a common finding. Prolactin is not the factor that enhances the hyperplastic phenotype in females while estrogen is a permissive factor. VEGF-A expression is increased in pituitaries from D2R KO. VEGF-A is expressed in follicle stellate cells. Because D2R receptors are found in lactotropes and not in follicle stellate cells, it may be inferred that a paracrine-derived factor from lactotropes is acting on follicle stellate cells to increase VEGF-A expression. VEGF-A does not induce pituitary cell proliferation, even though it enhances prolactin secretion. But it may act on adjacent endothelial cells and participate in the angiogenic process that increases the availability of different growth factors and mitogens. The D2R knockout mouse represents a unique animal model to study dopamine-resistant prolactinomas, and VEGF-A may be an alternative therapeutic target in this pathology.

Peliosis hepatis: spectrum of imaging findings.

OBJECTIVE: It is important to recognize the imaging characteristics of peliosis hepatis because peliotic lesions may mimic several different types of focal hepatic lesions CONCLUSION: We illustrate the spectrum of imaging findings of peliosis hepatis, including sonography, CT, MR, and angiography.