Results 111 to 120 of about 22,483 (254)

Polymorphism of the complement receptor 1 gene correlates with the hematologic response to eculizumab in patients with paroxysmal nocturnal hemoglobinuria

Haematologica, 2014
Complement blockade by eculizumab is clinically effective in hemolytic paroxysmal nocturnal hemoglobinuria. However, the response is variable and some patients remain dependent on red blood cell transfusions.
Tommaso Rondelli, Antonio M. Risitano, Régis Peffault de Latour, Michela Sica, Benedetta Peruzzi, Patrizia Ricci, Wilma Barcellini, Anna Paola Iori, Carla Boschetti, Veronica Valle, Veronique Frémeaux-Bacchi, Maria De Angioletti, Gerard Socie, Lucio Luzzatto, Rosario Notaro   +14 more
doaj   +1 more source

Paroxysmal Nocturnal Hemoglobinuria: Diagnostic Challenges in Pediatric Patient

Case Reports in Pediatrics, 2019
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening hematologic stem cell disorder characterized by hemoglobinuria, thrombosis, and tendency for bone marrow failure.
Dharshana Krishnaprasadh, Inna Kaminecki, Anna Sechser Perl, Jonathan Teitelbaum   +3 more
doaj   +1 more source

Ravulizumab (ALXN1210) in patients with paroxysmal nocturnal hemoglobinuria: results of 2 phase 1b/2 studies.

Blood Advances, 2018
Ravulizumab (ALXN1210), a humanized monoclonal antibody to complement component C5, was engineered from eculizumab to have a substantially longer terminal half-life, permitting longer dosing intervals for paroxysmal nocturnal hemoglobinuria (PNH ...
A. Röth, S. Rottinghaus, A. Hill, Eric S. Bachman, Jin Seok Kim, H. Schrezenmeier, L. Terriou, Á. Urbano-Ispizua, R. Wells, J. Jang, A. Kulasekararaj, J. Szer, R. Aguzzi, A. Damokosh, L. Shafner, J. W. Lee   +15 more
semanticscholar   +1 more source

Off-Pump Coronary Revascularization Using Bilateral Internal Thoracic Arteries in A Patient with Paroxysmal Nocturnal Hemoglobinuria: A Case Report

Brazilian Journal of Cardiovascular Surgery
Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-orphan disease. We report the first case in the literature of Off-Pump Coronary Revascularization Using Bilateral Internal Thoracic Arteries in a patient with paroxysmal nocturnal hemoglobinuria. A 36-
Juan Mariano Vrancic, Manuel Roque Cervetti, Julián Benavides, Daniel Navia   +3 more
doaj   +1 more source

Distribution of decay-accelerating factor in the peripheral blood of normal individuals and patients with paroxysmal nocturnal hemoglobinuria. [PDF]

, 1985
Taroh Kinoshita, M. Edward Medof, R Silber, Victor Nussenzweig   +3 more
openalex   +1 more source

Complement and inflammasome overactivation mediates paroxysmal nocturnal hemoglobinuria with autoinflammation.

Journal of Clinical Investigation, 2019
Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a clonal population of blood cells deficient in glycosylphosphatidylinositol (GPI)-anchored proteins, resulting from a mutation in the X-linked gene PIGA. Here we report on a set of patients in
B. Höchsmann, Y. Murakami, Makiko Osato, Alexej Knaus, M. Kawamoto, N. Inoue, T. Hirata, S. Murata, M. Anliker, T. Eggermann, M. Jäger, Ricarda Floettmann, Alexander Höllein, S. Murase, Yasutaka Ueda, J. Nishimura, Y. Kanakura, N. Kohara, H. Schrezenmeier, P. Krawitz, T. Kinoshita   +20 more
semanticscholar   +1 more source

Myeloproliferative neoplasms, thrombosis and paroxysmal nocturnal hemoglobinuria: Is this triad more frequent than we thought? [PDF]

, 2017
V Gaidano, Massimo Geuna, A Cignetti, Francesca Carnuccio, P. A. Bernabei, Nicola Santoro, Giuseppe Saglio, Piera Sivera   +7 more
openalex   +1 more source

Paroxysmal cold hemoglobinuria successfully treated with complement inhibition.

Blood Advances, 2019
This is the first-ever demonstration of successful treatment of paroxysmal cold hemoglobinuria using the complement inhibitor eculizumab.
Sarah A. Lau-Braunhut, H. Stone, G. Collins, S. Berentsen, B. Braun, M. Zinter   +5 more
semanticscholar   +1 more source

PAROXYSMAL HEMOGLOBINURIA [PDF]

American Journal of Diseases of Children, 1912
Charpentier's treatise on the various types of hemoglobinuria is so concise and reviews the literature so thoroughly to the date of its publication that it would be futile to go over the details here. He describes paroxysmal hemoglobinuria as follows: It usually attacks men (women are seldom affected of a pale and sallow complexion, who may or may not
openaire   +1 more source

Impaired phagocyte responses to lipopolysaccharide in paroxysmal nocturnal hemoglobinuria [PDF]

, 1993
J. Duchow, Arnaud Marchant, Alain Crusiaux, Cécile Husson, Cristina Alonso‐Vega, D De Groote, Paul Neve, Michel Goldman   +7 more
openalex   +1 more source