Results 41 to 50 of about 82,322 (154)

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

Treatment of paroxysmal nocturnal hemoglobinuria [PDF]

Blood, 1982
Patients with PNH may be treated with a number of known agents. As in all patients with a chronic disease, a regimen tolerable over a long period of time must be selected. Knowledge and anticipation of complications and their proper treatment are essential parts in the treatment.
openaire   +3 more sources

Expert Consensus on the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria (PNH): An Algorithmic Approach in an Era of New Treatments

European Journal of Haematology, EarlyView.
ABSTRACT Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by a defect of glycosylphosphatidyl‐anchored proteins, leading to an uncontrolled complement‐mediated hemolysis. The advent of complement inhibitors in clinical practice radically changed patients' outcomes and survival.
Bruno Fattizzo, Monia Marchetti, Elisa Cannizzo, Arianna Gatti, Massimo Geuna, Anna Paola Iori, Maddalena Raia, Simona Sica, Francesco Buccisano   +8 more
wiley   +1 more source

Short-term recurrent coronary artery thrombosis with acute myocardial infarction in a patient with aplastic anemia–paroxysmal nocturnal hemoglobinuria syndrome: a case report

Frontiers in Cardiovascular Medicine
BackgroundAcute myocardial infarction commonly occurs in patients with coronary artery disease, but rarely, it can develop under a hypercoagulable state.
Xue-Guo Fu, Yan-Hua Guo, Shi-Chao Wang, Wen-Quan Zhang   +3 more
doaj   +1 more source

Hematopoietic stem cell transplantation for paroxysmal nocturnal hemoglobinuria: long-term results of a retrospective study on behalf of the Gruppo Italiano Trapianto Midollo Osseo (GITMO)

Haematologica, 2010
Background Paroxysmal nocturnal hemoglobinuria is an acquired clonal disorder of the hemopoietic stem cells for which the only curative treatment is allogeneic hematopoietic stem cell transplantation.Design and Methods The aim of this retrospective study
Stella Santarone, Andrea Bacigalupo, Antonio M. Risitano, Elena Tagliaferri, Erminia Di Bartolomeo, Anna Paola Iori, Alessandro Rambaldi, Emanuele Angelucci, Alessandra Spagnoli, Federico Papineschi, Stefania Tamiazzo, Marta Di Nicola, Paolo Di Bartolomeo   +12 more
doaj   +1 more source

Australian consensus recommendations for the management of increased meningococcal infection risk in adults with neurological diseases treated with complement inhibitors

Internal Medicine Journal, EarlyView.
Abstract Complement inhibitor therapy carries a risk of serious infections, including meningococcal disease. Here we provide evidence‐based recommendations and expert consensus for immunisation and prophylactic treatment of patients receiving, or planning to receive, complement inhibitors for neurological conditions in the Australian setting.
Katherine A. Buzzard, Michael Barnett, Heidi Beadnall, Mirasol Forcadela, Lesley‐Ann Hall, Victoria G. Hall, Todd Hardy, Danny Hsu, Paul Kinsella, Mastura Monif, Sudarshini Ramanathan, Stephen W. Reddel, Sean Riminton, Douglas F. Johnson   +13 more
wiley   +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

Mapping of C5‐Blocking Monoclonal Antibodies Reveals New C5 Inhibitory Epitopes and Novel Modes of C5 Inhibition

Immunology, EarlyView.
We identify four distinct C5 inhibitory epitopes, including two novel antibodies that selectively block membrane attack complex (MAC) assembly while preserving C5 cleavage and C5a generation. These findings establish selective MAC inhibition as a new therapeutic strategy for complement‐mediated diseases.
Rebekah Sian Cooke, Kok Yung Lee, Georgina Elizabeth Menzies, Masashi Mizuno, Bryan Paul Morgan, Wioleta Milena Zelek   +5 more
wiley   +1 more source

Breakthrough Hemolysis in Paroxysmal Nocturnal Hemoglobinuria: Mechanistic Insights and Management Strategies

Transfusion, EarlyView.
Abstract Background Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder caused by somatic mutations in the PIGA gene, resulting in loss of glycosylphosphatidylinositol (GPI)‐anchored proteins, including the complement regulatory proteins, CD55 and CD59.
Ganesh Raman, Meghana Vivek, Rida Hasan, Siobán Keel, Laura Connelly‐Smith   +4 more
wiley   +1 more source

The small population of PIG-A mutant cells in myelodysplastic syndromes do not arise from multipotent hematopoietic stem cells

Haematologica, 2012
Background Patients with paroxysmal nocturnal hemoglobinuria harbor clonal glycosylphosphatidylinositol-anchor deficient cells arising from a multipotent hematopoietic stem cell acquiring a PIG-A mutation.
Jeffrey J. Pu, Rong Hu, Galina L. Mukhina, Hetty E. Carraway, Michael A. McDevitt, Robert A. Brodsky   +5 more
doaj   +1 more source