Granulocytic sarcoma of the small bowel, greater omentum and peritoneum associated with a CBFβ/MYH11 fusion and inv(16) (p13q22): a case report

<p>Abstract</p> <p>Introduction</p> <p>Granulocytic sarcoma (GS) is an extramedullary disease which is composed of immature myeloid cells or myeloblasts and usually occurs in association with acute myeloid leukemia (AML), as an initial presentation or a relapse. GS has been associated with various cytogenetic abnormalities, particularly with the t(8;21) translocation and less frequently the inv(16) type.</p> <p>Case presentation</p> <p>We present a rare case of GS of the small bowel, greater omentum and peritoneum, which caused obstruction, in a patient with AML associated with a CBFβ/MYH11 fusion gene and an inv(16) (p13q22). In this patient there was only mild myeloid hyperplasia in bone marrow aspiration but molecular analysis identified a CBFβ-MYH11 fusion and inv(16) (p13;q22).</p> <p>Conclusion</p> <p>Because of its nonspecific clinical and radiologic findings, this entity can be misdiagnosed and can mimic other solid neoplasms, making it a diagnostic challenge. In a GS with no or minimal morphological changes in bone marrow aspiration it is very important to perform a cytogenetic analysis to benefit from the diagnosis and therapeutic strategy.</p

Sarcoidosis sistémica con afectación gastrointestinal y hepática

Sarcoidosis is a systemic granulomatous disease of uncertain aetiology. It is diagnosed when non-necrotising granulomas are found in the absence of other autoimmune or infectious processes or external agents. The incidence of gastrointestinal involvement is estimated at 0.1-1.6%, while liver involvement occurs in 50-65% of cases, and may be subclinical or discovered in the study of liver enzyme abnormalities. We present the case of a 51-year-old man with postprandial satiety, epigastric pain and refractory ascites in the context of sarcoidosis with gastrointestinal, and hepatic involvement. He was treated with prednisone and azathioprine with subsequent clinical stabilisation.La sarcoidosis es una afectación granulomatosa sistémica de etiología incierta. Se diagnostica ante el hallazgo de granulomas no necrotizantes en ausencia de otros procesos autoinmunes, infecciosos o agentes externos. La incidencia de la afectación gastrointestinal se estima en un 0,1-1,6% mientras que la afectación hepática ocurre en el 50-65% de los casos, pudiendo ser subclínica o descubierta en el estudio de una alteración de las enzimas hepáticas. Presentamos el caso de un varón de 51 años con un cuadro de saciedad posprandial, dolor epigástrico y ascitis refractaria en contexto de una sarcoidosis con afectación gastrointestinal y hepática. Se inició tratamiento con prednisona y azatioprina con estabilización clínica posterior

Seasonal climate predictions in support of water reservoirs management in Spain

Póster presentado en: 1st Climateurope festival celebrado en Valencia, del 5 al 7 de abril de 2017.This work has been partially funded by the FP7 EUPORIAS projec

Congenital extrahepatic portosystemic shunts (Abernethy malformation): An international observational study

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach

Reseñas iberoamericanas

Iberoamerican ReviewsReseñas iberoamericana

Oxidative stress is a consequence, not a cause, of aluminum toxicity in the forage legume Lotus corniculatus

40 Pags., 4 Tabls., 9 Figs. The definitive version is available at: www3.interscience.wiley.com•Aluminum (Al) toxicity is a major limiting factor of crop production on acid soils, but the implication of oxidative stress in this process is controversial. A multidisciplinary approach was used here to address this question in the forage legume Lotus corniculatus.•Plants were treated with low Al concentrations in hydroponic culture, and physiological and biochemical parameters, together with semiquantitative metabolic and proteomic profiles, were determined.•The exposure of plants to 10 μM Al inhibited root and leaf growth, but had no effect on the production of reactive oxygen species or lipid peroxides. By contrast, exposure to 20 μM Al elicited the production of superoxide radicals, peroxide and malondialdehyde. In response to Al, there was a progressive replacement of the superoxide dismutase isoforms in the cytosol, a loss of ascorbate and consistent changes in amino acids, sugars and associated enzymes.•We conclude that oxidative stress is not a causative factor of Al toxicity. The increased contents in roots of two powerful Al chelators, malic and 2-isopropylmalic acids, together with the induction of an Al-activated malate transporter gene, strongly suggest that both organic acids are implicated in Al detoxification. The effects of Al on key proteins involved in cytoskeleton dynamics, protein turnover, transport, methylation reactions, redox control and stress responses underscore a metabolic dysfunction, which affects multiple cellular compartments, particularly in plants exposed to 20 μM Al.Joaquín Navascués was the recipient of a postdoctoral contract (JAE-CSIC program). This work was funded by the European Commission (FP6-2003-INCO-DEV2- 517617), the Ministry of Science and Innovation-Fondo Europeo de Desarrollo Regional (AGL2008-01298 and AGL2010-16515), and Gobierno de Aragón-Fondo Social Europeo (group A53).Peer reviewe