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Bosch A.,Unite de Transplantation Hepatique | Dumortier J.,Unite de Transplantation Hepatique | Maucort-Boulch D.,French National Center for Scientific Research | Scoazec J.-Y.,Service Central dAnatomie et de Cytologie Pathologiques | And 10 more authors.
Journal of Hepatology | Year: 2015

Background & Aims Recurrence of primary biliary cirrhosis (PBC) after liver transplantation (LT) is not rare and can occasionally lead to severe graft dysfunction and retransplantation. Ursodeoxycholic acid (UDCA) is a safe and effective treatment for PBC. However, whether preventive administration of UDCA after LT could lower the incidence of PBC recurrence is unknown. Methods Patients transplanted for PBC in five French and Swiss centers from 1988 to 2010 were included. Most patients from a single center received UDCA (10-15 mg/kg/d) preventively. Recurrence of PBC was histologically defined from biopsies routinely performed at 1, 5, 10, and 15 years of follow-up, and at any time when clinically indicated. Results A total of 90 patients with a 1-year minimum follow-up were studied retrospectively, including 19 (21%) patients receiving preventive UDCA. The mean follow-up was 12 years. Recurrence was diagnosed in 48 (53%) patients. The recurrence rates at 5, 10, and 15 years were 27%, 47%, and 61%, respectively. In a multivariate proportional hazards model adjusted for potential confounders and risk factors, preventive UDCA was the only factor affecting the risk of recurrence significantly (HR = 0.32; 95% CI: 0.11-0.91). The 5, 10, and 15-year rates of recurrence were 11%, 21%, and 40%, respectively, under preventive UDCA, and 32%, 53%, and 70%, respectively, without preventive UDCA. Seven patients with recurrence (15%) progressed to cirrhosis, requiring retransplantation in one. However, neither recurrence nor preventive UDCA had a significant impact on survival. Conclusions Preventive treatment with UDCA reduces the risk of PBC recurrence after LT.


We report the case of a 55-year-old male with renal failure as the initial manifestation of interstitial and focal infiltration of the kidneys by a small B-cell lymphoma. Since three years, this patient had a history of CLL with plasmocytic differenciation and was left untreated owing to stade A Binet classification. After chemotherapy, the lymphocytosis and the adenopathies disappear and the renal function improve. Infiltration of the kidneys by non-Hodgkin small B-cell lymphoma, including chronic lymphocytic leukaemia (CLL), is usually asymptomatic, fortuitously discovered at the time of an X-ray examination or at autopsy. Association with renal failure is extremely rare. We review the reported cases of renal failure associated with lymphomatous infiltration (13 cases of CLL and five cases of lymphoplasmocytic lymphoma kappa or lambda IgM), with the following conclusions: in most cases, renal insufficiency appears in a few months and significantly disappears after chemotherapy; the renal infiltrate is usually focal in lymphoplasmocytic lymphoma and rather massive and diffuse in CLL; the neoplastic feature of a small B-cell lymphoïd infiltrate may be difficult to determine: a poorly limited, monomorphous, CD20+ CD5+ lymphoid infiltrate is lymphomatous. In case of plasmocytic differenciation, it must be looked for kappa or lambda monotypy; the type of the lymphomatous infiltrate according to the WHO 2008 classification may be difficult to determine in a small sampling of renal tissue: the renal infiltrate must be compared, if possible, with a lymph node infiltrate. Owing to its bad prognosis, mantle cell lymphoma must be distinguished from other small B-cell lymphoma like CLL/small lymphocytic lymphoma, marginal zone lymphoma and lymphoplasmocytic lymphoma. © 2011 Association Socié té de né phrologie. Published by Elsevier Masson SAS. All rights reserved.


PubMed | Service de Transplantation Hepatique, Hopitaux Universitaires Of Geneva, French Institute of Health and Medical Research, Service dAnatomie et de Cytologie Pathologiques and 4 more.
Type: Journal Article | Journal: Journal of hepatology | Year: 2015

Recurrence of primary biliary cirrhosis (PBC) after liver transplantation (LT) is not rare and can occasionally lead to severe graft dysfunction and retransplantation. Ursodeoxycholic acid (UDCA) is a safe and effective treatment for PBC. However, whether preventive administration of UDCA after LT could lower the incidence of PBC recurrence is unknown.Patients transplanted for PBC in five French and Swiss centers from 1988 to 2010 were included. Most patients from a single center received UDCA (10-15 mg/kg/d) preventively. Recurrence of PBC was histologically defined from biopsies routinely performed at 1, 5, 10, and 15 years of follow-up, and at any time when clinically indicated.A total of 90 patients with a 1-year minimum follow-up were studied retrospectively, including 19 (21%) patients receiving preventive UDCA. The mean follow-up was 12 years. Recurrence was diagnosed in 48 (53%) patients. The recurrence rates at 5, 10, and 15 years were 27%, 47%, and 61%, respectively. In a multivariate proportional hazards model adjusted for potential confounders and risk factors, preventive UDCA was the only factor affecting the risk of recurrence significantly (HR=0.32; 95% CI: 0.11-0.91). The 5, 10, and 15-year rates of recurrence were 11%, 21%, and 40%, respectively, under preventive UDCA, and 32%, 53%, and 70%, respectively, without preventive UDCA. Seven patients with recurrence (15%) progressed to cirrhosis, requiring retransplantation in one. However, neither recurrence nor preventive UDCA had a significant impact on survival.Preventive treatment with UDCA reduces the risk of PBC recurrence after LT.


Candelier J.-J.,French Institute of Health and Medical Research | Candelier J.-J.,University Paris - Sud | Frappart L.,Service Central dAnatomie et de Cytologie Pathologiques | Diatta A.L.,Laboratoire Of Cytogenetique Et Of La Reproduction Et Service Dobstetrique | And 11 more authors.
Virchows Archiv | Year: 2013

Trophoblast cell adhesion and migration are carefully coordinated during normal placental development. We have compared the expression of three adhesion molecules, E-cadherin, β-catenin, and Lewis x, by immunohistochemistry during normal trophoblast differentiation, and in hydatidiform moles and choriocarcinomas. Both E-cadherin and β-catenin were expressed in normal placenta cytotrophoblast, and this expression decreased with trophoblast maturation. E-cadherin was mainly localized along the contact between cytotrophoblast and syncytiotrophoblast, which indicates its role in the differentiation of the syncytial layer. Lewis x disappeared progressively during differentiation of normal villous vessels, and was expressed in molar pregnancies. Interestingly, whereas choriocarcinomas were not, or poorly, stained, invasive hydatidiform moles (invHMs) strongly expressed Lewis x in vascular structures. This observation correlated well with E-cadherin and β-catenin expression and suggests that these three markers are associated with the invasive transformation. The presence of robust endothelial structures in invHMs could also explain their ability to maintain organized villous architecture (contrary to metastatic choriocarcinomas) during their invasion of extrauterine tissues such as the lung or the brain after dissemination through the blood flow. In our hands, Lewis x appeared to be a new, reliable marker that can be used to clearly distinguish invHMs from choriocarcinomas. © 2013 Springer-Verlag Berlin Heidelberg.


Candelier J.-J.,French Institute of Health and Medical Research | Candelier J.-J.,University Paris - Sud | Frappart L.,Service Central dAnatomie et de Cytologie Pathologiques | Yadaden T.,Center Hospitalier | And 4 more authors.
Pathology and Oncology Research | Year: 2013

Abnormal trophoblast differentiation is the main cause of gestational trophoblast diseases in the case of hydatidiform moles and choriocarcinomas. Here we investigated the expression patterns of two gene products, p16 and Bcl-2, implicated in cell cycle regulation and apoptosis, respectively, using immunohistochemistry during normal placenta differentiation, hydatidiform moles (partial, complete and invasive) and post-molar choriocarcinomas. The p16 protein shows a gradual expression in cytotrophoblast of normal villous, from a p16 weak proliferative phenotype to a p16 strong invasive phenotype reaching a maximum around 17 weeks of gestation. The expression pattern in cytotrophoblast was similar in moles in contrast to the villous mesenchyme of invasive moles where p16 was strongly expressed. Bcl-2 expression was syncytiotrophoblast specific in normal placenta and moles and increased gradually during normal differentiation. The results explain the persistence of normal and molar villous fragments during their development and their dramatic invasion in the uterine arteries in case of invasive moles. In choriocarcinomas the weak Bcl-2 expression is associated with weak p16 expression indicating a great apoptotic and proliferative potentials. The results suggest that strong p16 expression in the villous mesenchyme may be responsible in part of the morbidity of the moles, and the key of cancer progression in the choriocarcinomas would be a fast cell-cycle turnover. © 2012 Arányi Lajos Foundation.


Uriel A.,North Manchester General Hospital | Stow R.,North Manchester General Hospital | Johnson L.,North Manchester General Hospital | Varma A.,Hope Hospital | And 6 more authors.
Clinical Infectious Diseases | Year: 2010

We describe 3 individuals infected with human immunodeficiency virus with unusual focal brain syndromes; magnetic resonance imaging revealed "open-ring" pattern space occupying lesions. After deterioration while the patientswere receiving anti-Toxoplasma therapy, brain biopsy was performed, which revealed aggressive demyelination consistent with tumefactive demyelination. Treatment with high-dose steroids resulted in complete recovery in all cases. © 2010 by the Infectious Diseases Society of America. All rights reserved.


Bouazra H.,Service de Pneumo allergologie | Loukil M.,Service de Pneumo allergologie | Bouzaidi K.,Service de Radiologie | Douggaz A.,Service Central dAnatomie et de Cytologie Pathologiques | Ghrairi H.,Service de Pneumo allergologie
Revue des Maladies Respiratoires | Year: 2013

Endobronchial hamartochondroma is a form of rare benign tumour. Compared to those that occur in the lung parenchyma, the endobronchial form can potentially be managed by relatively conservative treatment involving per-endoscopic resection. Comment. - A 61-year-old patient had a dry cough and chest pain for 3 months. Their clinicalexamination was normal, but thoracic CT scan showed lingular collapse. Bronchoscopy revealedthe presence of a multilobar tumour occluding the orifice of the lingula bronchus. Bronchialbiopsies were consistent with the diagnosis of a hamartochondroma. Before the destruction ofany of the left upper lobe parenchyma, the tumor was resected surgically and the patient hadan uneventful postoperative course.Conclusion. - Endobronchial hamartochondroma support must be rapid to avoid irreversibleparenchymal consequences downstream obstruction involving an often mutilating surgery.Essentially endoscopic treatment should be the most conservative possible.© 2013 SPLF.


Angileri F.,Laval University | Roy V.,Laval University | Morrow G.,Laval University | Scoazec J.Y.,Service Central danatomie et de Cytologie Pathologiques | And 3 more authors.
Biochimica et Biophysica Acta - Molecular Basis of Disease | Year: 2015

Hereditary tyrosinemia type 1 (HT1) is the most severe inherited metabolic disease of the tyrosine catabolic pathway, with a progressive hepatic and renal injury and a fatal outcome if untreated. Toxic metabolites accumulating in HT1 have been shown to elicit endoplasmic reticulum (ER) stress response, and to induce chromosomal instability, cell cycle arrest and apoptosis perturbation. Although many studies have concentrated on elucidating these events, the molecular pathways responsible for development of hepatocellular carcinoma (HCC) still remain unclear. In this study the fah knockout murine model (fah-/-) was used to investigate the cellular signaling implicated in the pathogenesis of HT1. Fah-/- mice were subjected to drug therapy discontinuation (Nitisinone withdrawal), and livers were analyzed at different stages of the disease. Monitoring of mice revealed an increasing degeneration of the overall physiological conditions following drug withdrawal. Histological analysis unveiled diffuse hepatocellular damage, steatosis, oval-like cells proliferation and development of liver cell adenomas. Immunoblotting results revealed a progressive and chronic activation of stress pathways related to cell survival and proliferation, including several stress regulators such as Nrf2, eIF2α, CHOP, HO-1, and some members of the MAPK signaling cascade. Impairment of stress defensive mechanisms was also shown by microarray analysis in fah-/- mice following prolonged therapy interruption. These results suggest that a sustained activation of stress pathways in the chronic HT1 progression might play a central role in exacerbating liver degeneration. © 2015.


Diebold J.,Service Central dAnatomie et de Cytologie Pathologiques | Audouin J.,Service Central dAnatomie et de Cytologie Pathologiques | Tourneau A.L.,Service Central dAnatomie et de Cytologie Pathologiques | Molina T.J.,University of Paris Descartes
Revue Francophone des Laboratoires | Year: 2015

The name g€ histiocytosis' is given to an accumulation of histiocytes in a tissue or in an organ. The spleen is one of the organ in which histiocytosis are the most frequent. Splenic histiocytosis is responsible of the development of a splenomegaly. The diagnosis is based on the study of clinical and biological symptoms. Imaging technics demonstrate either a diffuse infiltration or masses. A splenectomy for diagnosis is often performed. Pathological study brings important arguments for the diagnosis of histiocytosis based on the cell morphology and phenotype (expression of CD68 and 163), for the diagnosis of the aetiology and the treatment.Three types of histiocytosis of the spleen can be described:reactive histiocytosis dues to various infections, parasitosis, dysimmune diseases, malignant haematologic diseases (with a peculiar type with hemophagocytosis, sometimes associated with an activated macrophage syndrome) or various metabolic diseases with lipidic histiocytosis;neoplastic histiocytosis as Langerhans cell histiocytosis or more aggressive tumours as true histiocytic sarcoma, Langerhans cell sarcoma, interdigitating cell sarcoma;finally, a variety of splenic histiocytosis of undetermined significance, for which more studies are needed. © 2015 Elsevier Masson SAS.


PubMed | Service Central danatomie et de Cytologie Pathologiques and Laval University
Type: Journal Article | Journal: Biochimica et biophysica acta | Year: 2015

Hereditary tyrosinemia type 1 (HT1) is the most severe inherited metabolic disease of the tyrosine catabolic pathway, with a progressive hepatic and renal injury and a fatal outcome if untreated. Toxic metabolites accumulating in HT1 have been shown to elicit endoplasmic reticulum (ER) stress response, and to induce chromosomal instability, cell cycle arrest and apoptosis perturbation. Although many studies have concentrated on elucidating these events, the molecular pathways responsible for development of hepatocellular carcinoma (HCC) still remain unclear. In this study the fah knockout murine model (fah(-/-)) was used to investigate the cellular signaling implicated in the pathogenesis of HT1. Fah(-/-) mice were subjected to drug therapy discontinuation (Nitisinone withdrawal), and livers were analyzed at different stages of the disease. Monitoring of mice revealed an increasing degeneration of the overall physiological conditions following drug withdrawal. Histological analysis unveiled diffuse hepatocellular damage, steatosis, oval-like cells proliferation and development of liver cell adenomas. Immunoblotting results revealed a progressive and chronic activation of stress pathways related to cell survival and proliferation, including several stress regulators such as Nrf2, eIF2, CHOP, HO-1, and some members of the MAPK signaling cascade. Impairment of stress defensive mechanisms was also shown by microarray analysis in fah(-/-) mice following prolonged therapy interruption. These results suggest that a sustained activation of stress pathways in the chronic HT1 progression might play a central role in exacerbating liver degeneration.

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