Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry [PDF]
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
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Paroxysmal Nocturnal Hemoglobinuria
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 +6 more sources
Paroxysmal nocturnal hemoglobinuria revealed by atypical thromboses: A case of cerebral, hepatic, and portal vein involvement leading to cirrhosis [PDF]
Paroxysmal nocturnal hemoglobinuria is a rare clonal stem cell disorder characterized by ongoing intravascular hemolysis, frequent bone marrow dysfunction, and a markedly elevated risk of thrombosis.
Majda Malghi, MD +6 more
doaj +2 more sources
Short-term recurrent coronary artery thrombosis with acute myocardial infarction in a patient with aplastic anemia–paroxysmal nocturnal hemoglobinuria syndrome: a case report [PDF]
BackgroundAcute myocardial infarction commonly occurs in patients with coronary artery disease, but rarely, it can develop under a hypercoagulable state.
Xue-Guo Fu +3 more
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Meningococcal Sepsis in Patient with Paroxysmal Nocturnal Hemoglobinuria during Pegcetacoplan Therapy [PDF]
Complement C5 inhibitors bring an increased risk for Neisseria infections. A novel complement C3 inhibitor, pegcetacoplan, was recently approved to treat paroxysmal nocturnal hemoglobinuria, a condition commonly treated with complement C5 inhibitors. We
Leo Starck +8 more
doaj +2 more sources
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
Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria
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 +1 more source
Paroxysmal Nocturnal Hemoglobinuria [PDF]
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
Eculizumab treatment: stochastic occurrence of C3 binding to individual PNH erythrocytes [PDF]
C5 blockade by eculizumab prevents complement-mediated intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH). However, C3-bound PNH red blood cells (RBCs), arising in almost all treated patients, may undergo extravascular hemolysis ...
A Hill +64 more
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Paroxysmal nocturnal hemoglobinuria [PDF]
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.
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