Results 11 to 20 of about 12,184 (217)

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

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

Real-World Effectiveness and Safety of Ravulizumab in Patients With Paroxysmal Nocturnal Hemoglobinuria: Evidence From the International PNH Registry. [PDF]

Am J Hematol
ABSTRACT Ravulizumab, a second‐generation complement component 5 inhibitor (C5i) derived from eculizumab, with improved pharmacokinetics, is the current standard‐of‐care treatment for patients with paroxysmal nocturnal hemoglobinuria (PNH), where available.
Röth A, Patriquin CJ, Szer J, Terriou L, Patel AS, Metzger J, Gustovic P, Nishimura JI, Brodsky RA.   +8 more
europepmc   +2 more sources

Danazol for paroxysmal nocturnal hemoglobinuria [PDF]

American Journal of Hematology, 1997
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem-cell disorder in which blood cells lack complement inhibiting membrane proteins, and become susceptible to complement-mediated injury, leading to chronic intravascular hemolysis and pancytopenia. Glucocorticoids have been a mainstay of therapy.
W J, Harrington, L, Kolodny, L L, Horstman, W, Jy, Y S, Ahn   +4 more
openaire   +2 more sources

Paroxysmal nocturnal hemoglobinuria [PDF]

Vnitřní lékařství, 2018
Paroxysmal nocturnal hemoglobinuria (PNH) results from a deficiency in inhibitors of activated complement. This lack leads to complement mediated intravascular hemolysis, to activation of coagulation system with increased risk of thrombotic complications and to various degree of bone marrow failure.
openaire   +3 more sources

Paroxysmal Nocturnal Hemoglobinuria [PDF]

Blood, 2014
AbstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias. The absence of two glycosylphosphatidylinositol (GPI)-anchored proteins, CD55 and CD59, leads to uncontrolled complement activation that accounts for hemolysis and other PNH ...
openaire   +2 more sources

The kidneys in paroxysmal nocturnal hemoglobinuria [PDF]

Blood, 1981
Long-term study of 21 PNH patients revealed an unexpectedly high incidence of functional and anatomic renal abnormalities. Most patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. Evaluation of renal function revealed hyposthenuria, abnormal tubular function, and declining creatinine clearance ...
D A, Clark, S A, Butler, V, Braren, R C, Hartmann, D E, Jenkins   +4 more
openaire   +3 more sources

Detection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats

Haematologica, 2009
Background The presence of paroxysmal nocturnal hemoglobinuria clones in the setting of aplastic anemia or myelodysplastic syndrome has been shown to have prognostic and therapeutic implications. However, the status of paroxysmal nocturnal hemoglobinuria
Sa A. Wang, Olga Pozdnyakova, Jeffrey L. Jorgensen, L. Jeffrey Medeiros, Dariusz Stachurski, Mary Anderson, Azra Raza, Bruce A. Woda   +7 more
doaj   +1 more source

Paroxysmal Nocturnal Hemoglobinuria

Current Opinion in Hematology, 2012
The aim is to report on recent observations related to the natural history of paroxysmal nocturnal hemoglobinuria (PNH) and to review new therapeutic strategies for controlling the hemolysis of PNH.This review focuses on studies designed to characterize the long-term outcome of patients with PNH treated with eculizumab and to define the relationship ...
  +9 more sources

Extravascular hemolysis and complement consumption in Paroxysmal Nocturnal Hemoglobinuria patients undergoing eculizumab treatment [PDF]

, 2017
Los datos asociados con este artículo están disponibles en: http://dx.doi.org/10.1016/j.imbio.2016.09.002.Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia characterized by complement-mediated intravascular hemolysis that is ...
Anter, Jaouad, Ataúlfo Gonzalez-Fernández, Fernando, Forés, Rafael, García, Sheila Pinto, López, Alicia, López-Trascasa, Margarita, Martin Merinero, Hector, Ojeda, Emilio, Rodríguez de Córdoba, Santiago, Subías Hidalgo, Marta, Villegas, Ana   +10 more
core   +2 more sources