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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 +4 more
doaj +7 more sources
Paroxysmal Nocturnal Hemoglobinuria: Biology and Treatment
Medicina (Lithuania), 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 +2 more
exaly +4 more sources
Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry [PDF]
Haematologica, 2014 Paroxysmal nocturnal hemoglobinuria is a rare, acquired disease associated with hemolytic anemia, bone marrow failure, thrombosis, and, frequently, poor quality of life.
Hubert Schrezenmeier +12 more
doaj +4 more sources
The prothrombotic state in paroxysmal nocturnal hemoglobinuria: a multifaceted source [PDF]
Haematologica, 2018 Paroxysmal nocturnal hemoglobinuria is a rare acquired hematologic disorder, the most serious complication of which is thrombosis. The increased incidence of thrombosis in paroxysmal nocturnal hemoglobinuria is still poorly understood, but unlike many ...
Barnaby Peacock-Young +4 more
doaj +2 more sources
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 +8 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 +3 more
doaj +7 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.
Hilda Ding, Michael Jaglal
+5 more sources
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 +3 more
doaj +1 more source
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 +3 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 +4 more
openaire +2 more sources