Results 41 to 50 of about 15,613 (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

Peripheral versus Marrow Lipidomics in Patients with Severe Aplastic Anemia: Potential Indicators for Early Immunosuppressive Treatment Response

Lipids, EarlyView.
ABSTRACT This study aimed to explore the differences of peripheral blood (PB) and bone marrow serum lipidomic profiles in severe aplastic anemia (SAA) patients and their significance in predicting earlier immunosuppressive therapy (IST) response. A cohort of 11 newly diagnosed SAA patients and 15 healthy controls were enrolled between June 2020 and ...
Zexing Sun, Yilei Hong, Yaonan Hong, Chuanao Xin, Qi Liu, Huijin Hu, Yingying Shen, Ying Chen, Shan Liu, Yiping Shen, Yuhong Zhou, Dijiong Wu   +11 more
wiley   +1 more source

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]

, 2014
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core   +1 more source

Paroxysmal nocturnal hemoglobinuria: Pandora’s box?

Journal of Mind and Medical Sciences, 2020
Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired non-malignant hematological disorder which affects the pluripotent hematopoietic stem cell.
Mihnea-Alexandru Găman, Iulia Ursuleac, Daniel Coriu   +2 more
doaj   +1 more source

Phase II study of the triple combination of rabbit ATG, ciclosporin and eltrombopag in patients with transfusion‐dependent aplastic anaemia: West Japan Hematology Study Group (W‐JHS) AA02 trial

British Journal of Haematology, EarlyView.
Summary The efficacy of a triple combination of rabbit anti‐human thymocyte immunoglobulin (rATG), ciclosporin and eltrombopag (EPAG) was prospectively evaluated in patients with severe or transfusion‐dependent non‐severe aplastic anaemia (SAA) across 29 institutions in Japan. Sixty patients were enrolled, of whom 48 had SAA. The primary end‐point, the
Fumi Nakamura, Ken Ishiyama, Ritsuro Suzuki, Hiroyuki Maruyama, Yasushi Onishi, Kensuke Usuki, Hideo Koh, Katsuto Takenaka, Ryunosuke Saiki, Seishi Ogawa, Kana Matsumoto, Takuto Takahashi, Kazuyoshi Hosomichi, Yoshitaka Zaimoku, Kenichi Yoshimura, Itaru Matsumura, Koichi Akashi, Shinji Nakao   +17 more
wiley   +1 more source

Hemoglobinuria paroxística nocturna: estudio de la enfermedad y sus tratamientos [PDF]

, 2018
Paroxysmal nocturnal hemoglobinuria (PNH), term established by Enneking in 1925, is a rare, clonal, and acquired disease of the hematopoietic stem cells.
Aparicio Morales, Paula Isabel
core  

Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria

Haematologica, 2012
Background In the era of eculizumab, identifying patients with paroxysmal nocturnal hemoglobinuria who may benefit from allogeneic stem cell transplantation is challenging.Design and Methods We describe the characteristics and overall survival of 211 ...
Régis Peffault de Latour, Hubert Schrezenmeier, Andrea Bacigalupo, Didier Blaise, Carmino A. de Souza, Stephane Vigouroux, Roelf Willemze, Louis Terriou, Andre Tichelli, Mohamad Mohty, Sophie de Guibert, Judith C. Marsh, Jakob Passweg, Jean Yves Mary, Gerard Socié   +14 more
doaj   +1 more source

Pregnancy in Classical Paroxysmal Nocturnal Hemoglobinuria and Aplastic Anemia–Paroxysmal Nocturnal Hemoglobinuria: A High-Risk Constellation

Frontiers in Medicine, 2020
Pregnancies in paroxysmal nocturnal hemoglobinuria (PNH) are associated with increased morbidity and mortality. Retrospective studies suggest that outcome has improved with the advent of the complement inhibitor eculizumab.
Ferras Alashkar, Fuat H. Saner, Colin Vance, Ute Schmücker, Dörte Herich-Terhürne, Ulrich Dührsen, Angela Köninger, Alexander Röth   +7 more
doaj   +1 more source

The PNH French Working Group Experience: Building a Strong Network of Cytometrists

International Journal of Laboratory Hematology, EarlyView.
ABSTRACT Introduction The PNH working group was created in 2010 to initiate a workshop on testing white blood cells to share international recommendations. Methods Thirty‐five French and Belgian laboratories equipped with 2‐ or 3‐laser cytometers applied three different panels with or without FLAER reagent in 305 samples.
Orianne Wagner‐Ballon, Magali Le Garff‐Tavernier, Rémi Lestestu, Bernard Drenou, Agathe Debliquis, the PNH French Working Group «CytHem‐HPN», Chantal Fossat, Claire Loosen, Bernard Chatelain, Marie Loosveld   +9 more
wiley   +1 more source

Aki associated with macroscopic glomerular hematuria: Clinical and pathophysiologic consequences [PDF]

, 2012
Hematuria is a common finding in various glomerular diseases. This article reviews the clinical data on glomerular hematuria and kidney injury, as well as the pathophysiology of hematuria-associated renal damage.
Alberto Ortiz, August, Catalina Martín-Cleary, Clark, Clarkson, Cleary, Delclaux, Eduardo Gutiérrez, Feith, Fogazzi, Hebbel, Heyman, Hill, Hirsch, Honda, Jesús Egido, Juan Antonio Moreno, Kincaid-Smith, Kincaid-Smith, Luis Miguel Blanco-Colio, Manuel Praga, Nath, Nath, Ortiz de Montellano, Oscar Toldos, Pace-Asciak, Praga, Reeder, Shah, Vaziri   +29 more
core   +2 more sources