نتایج جستجو برای: pnh

تعداد نتایج: 752  

Journal: :Genetics and molecular research : GMR 2015
F Fonseca Coelho F Loli Guimarães W L Ribeiro Cabral P G Oliveira Salles E Cueva Mateo L Mendes Nogueira e Nogueira C E Corradi Fonseca K Braga Gomes

We evaluated the expression of the PCA3 gene in urine from patients with nodular hyperplasia/benign prostatic hyperplasia (PNH) or adenocarcinoma type prostate cancer (PCa).The study included 59 men: 22 with PCa, 26 with PNH, and 11 with no alterations (controls). Patients' urine was collected following prostatic massage and quantified by quantitative real-time PCR for prostate cancer antigen 3...

2016
Eitan FIBACH Mutaz DANA Eliezer A. RACHMILEWITZ

Objectives We studied the involvement of serum-complement (C') and oxidative stress in the hemolytic anemias Paroxysmal Nocturnal Hemoglobinuria (PNH) and Auto-Immune Hemolytic Anemia (AIHA), and the effect of Fermented Papaya Preparation (FPP), an antioxidant-containing yeast fermentation product of Carica papaya Linn. Methodology Normal human red blood cells (RBC) treated with the sulphydryl ...

Journal: :Blood 1997
K Horikawa H Nakakuma T Kawaguchi N Iwamoto S Nagakura T Kagimoto K Takatsuki

Bone marrow (BM) hypoplasia is a major cause of death in paroxysmal nocturnal hemoglobinuria (PNH). However, little is known about the molecular events leading to the hypoplasia. Considering the close pathologic association between PNH and aplastic anemia (AA), it is suggested that a similar mechanism operates in the development of their BM failure. Recent reports have indicated apoptosis-media...

2016
Mehmet Sinan Dal Abdullah Karakuş Mehmet Önder Ekmen Orhan Ayyildiz

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder characterized by intravascular hemolysis. Real-world experience of PNH management is largely unreported. A retrospective analysis was undertaken based on medical records from six patients with PNH [two with aplastic anemia (AA)] treated at our center, Dicle University, Turkey. Diagnosis was based on granulocyte PNH clones, ra...

Journal: :Cytometry. Part B, Clinical cytometry 2010
Michael J Borowitz Fiona E Craig Joseph A Digiuseppe Andrea J Illingworth Wendell Rosse D Robert Sutherland Carl T Wittwer Stephen J Richards

BACKGROUND Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by a somatic mutation in the PIGA gene, leading to a deficiency of proteins linked to the cell membrane via glycophosphatidylinositol (GPI) anchors. While flow cytometry is the method of choice for identifying cells deficient in GPI-linked proteins and is, therefore, necessary for the d...

Journal: :Jornal Brasileiro De Patologia E Medicina Laboratorial 2021

ABSTRACT Introduction: The paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease of the hematopoietic stem cells, and it clinically characterized by chronic intravascular hemolysis, bone marrow failure hypercoagulability leading to thrombosis. It a rare disorder cells that occurs due somatic mutation in gene phosphatidylinositol glycan class A (PIG-A). Objective: Here we revie...

Journal: :Blood 2007
Peter Hillmen Petra Muus Ulrich Dührsen Antonio M Risitano Jörg Schubert Lucio Luzzatto Hubert Schrezenmeier Jeffrey Szer Robert A Brodsky Anita Hill Gerard Socié Monica Bessler Scott A Rollins Leonard Bell Russell P Rother Neal S Young

Hemolysis and hemoglobinemia contribute to serious clinical sequelae in hemolytic disorders. In paroxysmal nocturnal hemoglobinuria (PNH) patients, hemolysis can contribute to thromboembolism (TE), the most feared complication in PNH, and the leading cause of disease-related deaths. We evaluated whether long-term treatment with the complement inhibitor eculizumab reduces the rate of TE in patie...

2006
Anita Hill Simon H. Ridley Dirk Esser Rodney G. Oldroyd Matthew J. Cullen Paula Kareclas Seán Gallagher Geoffrey P. Smith Stephen J. Richards Jennifer White Richard A. G. Smith Peter Hillmen

Paroxysmal nocturnal hemoglobinuria (PNH) results from the expansion of a hematopoietic clone that is deficient in glycosylphosphatidylinositol-anchored molecules. PNH is characterized by chronic hemolysis with acute exacerbations due to the uncontrolled activity of complement on PNH cells, which lack the inhibitor of homologous complement, CD59. Symptoms include severe fatigue, hemoglobinuria,...

2013
Kanjaksha Ghosh Manisha Madkaikar Maya Gupta Farah Jijina

OBJECTIVE The responses of 32 patients with paroxysmal nocturnal hemoglobinuria (PNH) were assessed after the patients were put on various combinations of danazol, prednisolone, and cyclosporine. MATERIALS AND METHODS Nineteen males and 13 females aged between 14 and 60 years with confirmed diagnosis of PNH were treated with danazol (4), danazol + cyclosporine (7), cyclosporine (1), and predn...

Journal: :Blood 1995
R E Ware W F Rosse S E Hall

The hematologic disorder paroxysmal nocturnal hemoglobinuria (PNH) occurs following an acquired somatic mutation in the Piga gene within a bone marrow stem cell. The progeny of this mutated cell cannot synthesize glycosylphosphatidylinositol (GPI) anchors, with a resultant deficiency in surface expression of all GPI-linked proteins. The protean clinical manifestations of PNH presumably result f...

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