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 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.

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 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.

Quiste epitelial esplénico y peliosis hepática: una infrecuente asociación / Splenic epithelial cyst and hepatic peliosis: an infrequent association

Se describe el caso de una mujer de 58 años de edad con un quiste epitelial esplénico asociado a peliosis hepática, constituyendo una relación no reportada en la literatura médica hasta la actualidad (AU)

This paper reports on a 58-year female patient with a splenic epithelial cyst associated to hepatic peliosis a relation not being reported on in medical literature as of yet (AU)

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: report of one case]. / Peliosis hepática focal: Caso clínico radiológico.

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 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.

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 associated with lymphoplasmacytic lymphoma: an autopsy case report.

A 72-year-old man with no previous history of liver disease was admitted to our university hospital with severe dyspnea, edema of the lower limbs, and weight loss. Within a few days of hospitalization, he died due to severe bleeding in the upper digestive tract. At autopsy, the liver displayed typical gross features of peliosis hepatis. In addition, a diffuse infiltration of liver, spleen, bone marrow, and lymph nodes by lymphoplasmacytic lymphoma was disclosed by light microscopy. In the liver, the neoplastic cells partially filled the peliotic cavities. Peliosis hepatis is a rare liver disease characterized by multiple blood-filled, dilated cavities within the liver parenchyma. Association of lymphoplasmacytic lymphoma and peliosis hepatis has rarely been reported in the literature. The pathologic findings of such an unusual association and a review of the literature are presented.

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.