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Paroxysmal Nocturnal Hemoglobinuria

Journal of Nepal Medical Association, 2005
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic disorder of acquired origin and is clinically manifested by chronic hemolysis, thromboses in various sites, and bone marrow failure.
Buddhi P Paudyal, M Zimmerman, A Karki, H Neupane, G Kayastha   +4 more
doaj   +6 more sources

Pathogenesis of Paroxysmal Nocturnal Hemoglobinuria.

Blood
Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant clonal hematopoietic disorder. There are two components to the pathogenesis of PNH: (i) a mutant stem cell, (ii) expansion of the mutant clone. Component (i) is straightforward: there is almost
L. Luzzatto, S. Nakao
semanticscholar   +3 more sources

Pegcetacoplan for Paroxysmal Nocturnal Hemoglobinuria.

Blood, 2022
Approximately a third of patients with paroxysmal nocturnal hemoglobinuria (PNH) remain transfusion dependent or have symptomatic anemia despite treatment with a C5 inhibitor.
G. Gerber, R. Brodsky
semanticscholar   +3 more sources

Managing Fatigue in Patients with Paroxysmal Nocturnal Hemoglobinuria: A Patient-Focused Perspective

Journal of Blood Medicine, 2022
The most frequently reported symptom in patients with paroxysmal nocturnal hemoglobinuria (PNH), a disease characterized by complement mediated hemolysis and chronic anemia, is “fatigue”.
Esther Natalie Oliva, Bruno Fattizzo, Wilma Barcellini   +2 more
exaly   +2 more sources

Paroxysmal Nocturnal Hemoglobinuria: Biology and Treatment

Medicina, 2023
Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal hematopoietic disorder characterized by the lack of glycosylphosphatidylinositol-anchored proteins (GPI-APs) as a consequence of somatic mutations in the phosphatidylinositol glycan ...
Carlos Bravo-Perez, Luca Guarnera, Nakisha D. Williams, Valeria Visconte   +3 more
doaj   +2 more sources

Evolutionary dynamics of paroxysmal nocturnal hemoglobinuria. [PDF]

PLoS Computational Biology, 2018
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal blood disorder characterized by hemolysis and a high risk of thrombosis, that is due to a deficiency in several cell surface proteins that prevent complement activation.
Nathaniel Mon Père, Tom Lenaerts, Jorge M Pacheco, David Dingli   +3 more
doaj   +6 more sources

Herlyn Werner Wunderlich Syndrome Presenting with Ischemic Stroke due to Suspected Paroxysmal Nocturnal Hemoglobinuria: A Case Report

Journal of Nepal Medical Association, 2021
Paroxysmal nocturnal hemoglobinuria can rarely present as cerebral ischemia and stroke due to arterial thrombosis. However, it should be considered in a young patient with bone marrow failure features, systemic thromboses, and hemolysis.
Ayushma Acharya, Prajwala Yogi, Pramod Singh, Tulsi Ram Bhattarai   +3 more
doaj   +1 more source

Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria

Hematology, Transfusion and Cell Therapy, 2021
Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and life-threatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure.
Rodolfo D. Cançado, Aderson da Silva Araújo, Alex Freire Sandes, Celso Arrais, Clarisse Lopes de Castro Lobo, Maria Stella Figueiredo, Sandra Fátima Menosi Gualandro, Sara Teresinha Olalla Saad, Fernando Ferreira Costa   +8 more
doaj   +1 more source

Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria.

New England Journal of Medicine, 2021
BACKGROUND Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease characterized by chronic complement-mediated hemolysis. C5 inhibition controls intravascular hemolysis in untreated PNH but cannot address extravascular hemolysis ...
P. Hillmen, J. Szer, I. Weitz, A. Röth, B. Höchsmann, J. Panse, K. Usuki, M. Griffin, J. Kiladjian, Carlos M. de Castro, H. Nishimori, L. Tan, M. Hamdani, Pascal Deschatelets, C. Francois, F. Grossi, T. Ajayi, A. Risitano, Régis Peffault de la Tour   +18 more
semanticscholar   +1 more source

Clinically important change for the FACIT-Fatigue scale in paroxysmal nocturnal hemoglobinuria: a derivation from international PNH registry patient data

Journal of Patient-Reported Outcomes, 2023
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare blood disease that leads to breakdown of the body’s red blood cells in the blood vessels (intravascular hemolysis). Many people with PNH have fatigue, which consists of tiredness and weakness.
D. Cella, P. Johansson, Yasutaka Ueda, I. Tomazos, Philippe Gustovic, Alice Wang, Ami S. Patel, H. Schrezenmeier   +7 more
semanticscholar   +1 more source