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 hepatoesplénica por enfermedad por arañazo de gato. Reporte de caso / Hepatosplenic peliosis by cat scratch disease: report of a case

Se presenta el caso de una paciente mujer de 73 años que acude por disnea progresiva, fiebre diurna, hiporexia y cefalea de diez días de evolución. Antecedente de neoplasia ovarica, gastritis crónica y tromboembolia pulmonar. La fiebre continuó a pesar de tratamiento antibiótico convencional. Al examen, se identificó compromiso pulmonar y adenopatías inguinales, estas últimas corroboradas por ecografía de partes blandas. Los cultivos realizados fueron negativos y el estudio radiológico mostró compromiso pleuroparenquimal en base de pulmón izquierdo. La tomogragía abdominal evidenció hepatoesplenomegalia con hipodensidades múltiples (peliosis); la serología fue compatible con Bartonella henselae. La paciente evolucionó favorablemente luego de 72 horas de iniciado en tratamiento con doxiciclina a 100 mg cada doce horas.

This is the case of a 73-year-old female patient who presented with progressive dyspnea, diurnal fever, hyporexia and headache lasting for 10 days. She had a past medical history of an ovarian neoplasia, chronic gastritis and pulmonary thromboembolism. Fever continued despite conventional antibiotic treatment. Her physical examination revealed pulmonary involvement and bilateral inguinal adenopathy; the latter was corroborated with soft tissue ultrasound. Cultures performed were reported as negative and a chest X-ray film showed pleuro-parenchymal involvement in the left pulmonary base. An abdominal CT scan showed hepatosplenomegaly with multiple hypodense images (peliosis); and serological studies were compatible with Bartonella henselae. Doxyciclin 100 mg every 12 hours was started, and the patient started recovering uneventfully 72 hours after therapy was instituted.

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 type of peliosis hepatis

No abstract available.

Primary hepatic angiosarcoma: a clinical and pathological analysis / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathological characteristics, differential diagnosis, and prognosis of primary hepatic angiosarcoma, and to review the literature.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Peliosis hepatoesplénica: informe de un caso y revisión de la literatura / Hepatosplenic peliosis: a case report and a literature review

La peliosis es una entidad benigna muy rara, que se caracteriza por cavidades repletas de sangre dentro de órganos sólidos, principalmente en el sistema reticuloendotelial. La peliosis puede también afectar otros órganos como pulmones, pleura, riñones, glándulas suprarrenales y estómago. Las lesiones pueden ser únicas o múltiples y de tamaño variable. La etiología es ignorada, pero se ha relacionado con la ingestión o contacto con fármacos y tóxicos diversos como esteroides y anticonceptivos orales, asociándose a enfermedades tumorales hematológicas y a enfermedades infecciosas, en particular a la tuberculosis; en algunos casos es idiopática. Presentamos el caso de una mujer de 60 años con antecedentes de hipertensión arterial. La exploración física y los estudios de imagen evidenciaron una hepatomegalia nodular gigante sin esplenomegalia. Los exámenes de laboratorio indicaron trastornos propios de insuficiencia hepática y la paciente fallece por shock hipovolémico y hemoperitoneo. El estudio necrópsico determinó la existencia de una peliosis hepatoesplénica parenquimatosa “mayor” de etiología idiopática. Se lleva a cabo una revisión de la literatura, señalando aspectos relacionados con historia, patogénesis, etiología, aspectos clínicos, diagnósticos y terapéuticos.

Peliosis is a very rare benign entity which is characterized by blood filled cavities filled within solid organs. They occur primarily in the mononuclear phagocyte system although peliosis can also affect organs such as the lungs, pleura, kidneys, adrenal glands and stomach. Only one lesion or multiple lesions may occur, and their size is also variable. The etiology is unknown but is related to ingestion of, or direct contact with, drugs and various toxic substances such as steroids and oral contraceptives. It is associated with hematologic cancers and infectious diseases, particularly tuberculosis. In some cases it is idiopathic. We present the case of a 60 years old woman with a history of hypertension. Physical examination and various imaging studies showed a giant nodular hepatomegaly without splenomegaly. Laboratory studies showed disorders due to liver failure. The patient finally died of hypovolemic shock and hemoperitoneum. An autopsy determined the presence of parenchymal hepatosplenic peliosis of other than idiopathic etiology. A literature review highlighting the history, pathogenesis, etiology, clinical aspects, diagnosis and treatment of hepatosplenic peliosis is included.

A Case of Peliosis Hepatis Mimicking Metastatic Lesions of Increasing Size

Peliosis hepatis, an uncommon vascular condition, is characterized by multiple blood-filled cavities distributed throughout the liver. Computed tomography and magnetic resonance imaging findings of peliosis hepatis are nonspecific. A 40-year-old woman presented with multiple hepatic cystic masses. Two years later, the number and sizes of the masses had increased. We suspected metastatic hepatic disease and performed a liver biopsy. Histological examination revealed dilatation of hepatic sinusoids and multiple blood-filled cavities throughout the liver parenchyma. Thus, a diagnosis of peliosis hepatis was confirmed.

Peliosis hepática focal: Caso clínico radiológico / Peliosis hepatis: Report of one case

Peliosis hepatis is a rare vascular condition of the liver characterized by the presence of cystic blood-fi lled cavities distributed randomly throughout the liver parenchyma. We report a 46 year old female, with dyspepsia. An abdominal Doppler ultrasound showed a hyper vascularized solid liver nodule of 5 cm diameter. Magnetic resonance imaging and CT scan showed the same hyper vascularized nodule. With the possible diagnoses of primary hepatocellular carcinoma or focal nodular hyperplasia, the patient was subjected to an excision of hepatic segment VI, where the nodule was located. The pathological diagnosis of the surgical piece was a peliosis hepatis.

Peliosis Hepatis Cured with Anti-tuberculosis Medication in a Hemodialysis Patient / 대한신장학회지

Peliosis hepatis is an uncommon condition of blood-filled cystic cavities in the liver. Although it is difficult to distinguish this condition from hepatic malignancy or abscess in computed tomography (CT), differential diagnosis is important because it doesn't need further evaluation or treatment such as liver biopsy or surgery. We report a case of peliosis hepatis in a patient with active pulmonary tuberculosis in hemodialysis patient. A 39-year-old man receiving hemodialysis for 3 months was admitted because of fever. Chest computed tomography (CT) showed multiple necrotic lymphadenopathies and nodular lesion in right upper lobe of the lung suggesting active pulmonary tuberculosis. Three low attenuated lesions were shown in both hepatic lobes in abdominal CT. Liver biopsy was performed. The histopathologic diagnosis of peliosis hepatis in the liver was made by a blood-filled space with fibrin and hemorrhage. After anti-tuberculosis therapy, hepatic low attenuated lesions disappeared.

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 / 대한간학회지

No abstract available.

Peliosis Hepatis with Hemorrhagic Necrosis and Rupture: a Case Report with Emphasis on the Multi-Detector CT Findings

We report here on an uncommon case of peliosis hepatis with hemorrhagic necrosis that was complicated by massive intrahepatic bleeding and rupture, and treated by emergent right lobectomy. We demonstrate the imaging findings, with emphasis on the triphasic, contrast-enhanced multidetector CT findings, as well as reporting the clinical outcome in a case of peliosis hepatis with fatal hemorrhage.

Focal Peliosis Hepatis Mimicking Hepatocellular Carcinoma / 대한간학회지

No abstract available.

Peliosis hepática: abdomen agudo como forma de presentación / Hepatic peliosis: accute abdomen as presentation

Introducción: la peliosis hepática es una entidad de muy baja frecuencia, que se caracteriza por múltiples quistes llenos de sangre.Objetivo: presentación de caso y revisión bibliográfica.Lugar de aplicación: Sanatorio polivalente de alta complejidad.Material y métodos: presentación de caso. Revisión de la literatura.Caso Clínico: varón de 74 años, con diagnóstico anatomopatológico de peliosis hepática, con abdomen agudo.Discusión: Esta patología puede presentarse como abdomen agudo, pero su baja frecuencia contribuye a que no se tenga en cuenta en los diagnósticos diferenciales.Conclusiones: la peliosis hepática es una entidad muy poco frecuente. Cuando se presenta en forma de abdomen agudo su resolución debe ser inmediata, ya que compromete la vida del paciente.

Reporte de un caso de angiomatosis bacilar en un niño con infección por VIH / Bacillary angiomatosis in a child with HIV infection

Se reporta el caso de un lactante de 1 año y 2 meses, con serología positiva para VIH, natural y procedente de la Merced, que presentó cuadro de angiomatosis bacilar y peliosis, confirmada por anatomía patológica. El paciente respondió a la terapia convencional con eritromicina.

Peliosis Hepatis Mimicking Single Hepatic Mass: A Case Report

Peliosis hepatis is a rare disease characterized by cystic hepatic sinusoidal dilatation and the presence of multiple blood-filled spaces in the hepatic parenchyma. In most cases, multiple lesions and individual cysts not exceeding 1cm in diameter occur. We report a case in which the condition occurred in a 33-year-old woman who presented with general weakness and in whom a 3.5 cm-sized single hepatic mass was discovered incidentally at ultrasonography. The radiologic features mimicked those of a signle hepatic mass.

Virus TT (TTV) ¿Es un verdadero causante de hepatis? / Is TT virus (TTV) a true hepatitis virus cause?

El virus TT (TTV), se detectó en sangre de tres pacientes con hepatitis postransfusional que eran negativos para todos los marcadores de virus de hepatitis conocidos. Los títulos de ADN viral estaban correlacionados con los niveles de aminotransferas séricas (ALT). Además se aisló el virus de hígado con títulos iguales o mayores a los sueros correspondientes. Posteriormente se comprobó que la mayoría de las personas TTV positivas no presentan evidencias bioquímicas o histológicas de enfermedad hepática. TTV tiene una distribución mundial, con una alta prevalencia en la población general, y existen evidencias de que es transmisible a través de transfusiones sanguíneas, por vía entérica y de madre a hijo. Existe una importante cantidad de estudios que sugieren que este virus no es una causa significativa de enfermedad hepática crónica o aguda y hasta ahora no se ha descripto ninguna asociación de la infección con otras enfermedades.