Results 31 to 40 of about 8,824 (214)

Paroxysmal nocturnal hemoglobinuria clones in severe aplastic anemia patients treated with horse anti-thymocyte globulin plus cyclosporine

Haematologica, 2010
Background Clones of glycosylphosphatidylinositol-anchor protein-deficient cells are characteristic in paroxysmal nocturnal hemoglobinuria and are present in about 40–50% of patients with severe aplastic anemia.
Phillip Scheinberg, Michael Marte, Olga Nunez, Neal S. Young   +3 more
doaj   +1 more source

COVID‐19 infection presenting as paroxysmal nocturnal hemoglobinuria

Clinical Case Reports, 2021
A diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) was elicited during acute COVID‐19 infection. COVID‐19 spike proteins trigger the alternative pathway of complement. Acute SARS‐CoV‐2 infection possibly expanded an existing PIG‐A mutation.
Adam Hines, Nausheen Hakim, Jacqueline Barrientos   +2 more
doaj   +1 more source

Technical advances in flow cytometry-based diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria

Einstein (São Paulo)
Objective: To discuss the implementation of technical advances in laboratory diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria for validation of high-sensitivity flow cytometry protocols. Methods: A retrospective study based on analysis of
Rodolfo Patussi Correia, Laiz Cameirão Bento, Ana Carolina Apelle Bortolucci, Anderson Marega Alexandre, Andressa da Costa Vaz, Daniela Schimidell, Eduardo de Carvalho Pedro, Fabricio Simões Perin, Sonia Tsukasa Nozawa, Cláudio Ernesto Albers Mendes, Rodrigo de Souza Barroso, Nydia Strachman Bacal   +11 more
doaj   +1 more source

Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience

Haematologica, 2011
Background Pregnancy in women with paroxysmal nocturnal hemoglobinuria is rare, with few reports on maternal and fetal mortality rates.Design and Methods A specific questionnaire designed to solicit data on pregnancies in women with paroxysmal nocturnal ...
Sophie de Guibert, Régis Peffault de Latour, Nathalie Varoqueaux, Hélène Labussière, Bernard Rio, Dominique Jaulmes, Jean-Richard Eveillard, Stéphanie Dulucq, Anne-Marie Stoppa, Didier Bouscary, François Girodon, Bernard Bonnotte, Djamila Laskri, Gérard Socié, Thierry Lamy   +14 more
doaj   +1 more source

Thrombolytic therapy is effective in paroxysmal nocturnal hemoglobinuria: a series of nine patients and a review of the literature

Haematologica, 2012
Background Thrombosis is the major risk factor for death in patients with paroxysmal nocturnal hemoglobinuria. Previous case reports indicate that venous thrombosis in patients with paroxysmal nocturnal hemoglobinuria is amenable to thrombolysis.Design ...
David J. Araten, Rosario Notaro, Howard T Thaler, Nancy Kernan, Farid Boulad, Hugo Castro-Malaspina, Trudy Small, Andromachi Scaradavou, Heather Magnan, Susan Prockop, Sara Chaffee, Jason Gonsky, Raymond Thertulien, Roberto Tarquini, Lucio Luzzatto   +14 more
doaj   +1 more source

Very severe aplastic anemia in an 80‐year‐old man

Clinical Case Reports, 2021
Although the patient with very severe aplastic anemia might be a fit elderly receiving standard therapy, there are factors which contribute to an adverse outcome such as severity of pancytopenia, absence of minor paroxysmal nocturnal hemoglobinuria clone
Ganesh Kasinathan, Bee Sun Lee, Jameela Sathar   +2 more
doaj   +1 more source

Model-Informed Precision Dosing of Eculizumab in Patients with Paroxysmal Nocturnal Hemoglobinuria. [PDF]

Clin Pharmacokinet
Contains fulltext : 322381.pdf (Publisher’s version ) (Open Access)BACKGROUND AND OBJECTIVE: Eculizumab is an expensive therapeutic monoclonal antibody inhibiting complement C5 and approved for various indications, including the rare ...
Ter Avest M, Langemeijer SMC, van den Heuvel LPWJ, Baas LM, van de Kar NCAJ, Ter Heine R.   +5 more
europepmc   +2 more sources

Effectiveness of Iptacopan Versus C5 Inhibitors in Complement Inhibitor‐Naive Patients With Paroxysmal Nocturnal Haemoglobinuria

eJHaem
Background Paroxysmal nocturnal haemoglobinuria (PNH) is characterised by haemolytic anaemia, bone marrow failure and thrombosis. The single‐arm phase 3 APPOINT‐PNH trial (NCT04820530) investigating iptacopan monotherapy in complement inhibitor‐naive ...
Matthew Holt, Richard J. Kelly, Jilles M. Fermont, Soudeh Ansari, Marion Dahlke, Isabelle Brindel, Lynda Maafa, Flore Sicre deFontbrune, Régis Peffault deLatour   +8 more
doaj   +1 more source

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

Analysis of the Recovery of Immune Status in the Patients With Paroxysmal Nocturnal Hemoglobinuria After Treatment With C5 Complement Inhibitor

Journal of Clinical Laboratory Analysis, EarlyView.
We retrospectively evaluated the impact of C5 complement inhibition on immune cell profiles and recovery in 27 PNH patients; both CD4+ and CD8+ T cells exhibited partial recovery, while B cell counts increased but remained below normal levels. In contrast, Treg cells were significantly reduced in PNH patients and showed no notable change post‐treatment.
Hui Liu, Mengting Che, Liyan Li, Honglei Wang, Chaomeng Wang, Junshu Wu, Yingying Chen, Zhaoyun Liu, Lijuan Li, Rong Fu   +9 more
wiley   +1 more source