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
doaj +4 more sources
The prothrombotic state in paroxysmal nocturnal hemoglobinuria: a multifaceted source [PDF]
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
Paroxysmal nocturnal hemoglobinuria and telomere length predicts response to immunosuppressive therapy in pediatric aplastic anemia [PDF]
Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length
Atsushi Narita +21 more
doaj +2 more sources
Evaluating the treatment of patients with paroxysmal nocturnal hemoglobinuria in Brazil: disparities in access and treatment persistence [PDF]
Background: Paroxysmal nocturnal hemoglobinuria is a rare, life-threatening disease that significantly affects quality of life. Despite therapeutic advances, challenges like treatment burden and limited care access persist.
Rodolfo Delfini Cançado +10 more
doaj +2 more sources
Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil [PDF]
BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best
Lacy Cardoso de Brito Junior +5 more
doaj +2 more sources
Global Epidemiology of Paroxysmal Nocturnal Hemoglobinuria: A Systematic Literature Review [PDF]
Background Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematological disorder characterized by chronic intravascular hemolysis and an increased risk of thrombosis. A systematic synthesis of disease epidemiology is essential to understand
Olessia Zorina +6 more
doaj +2 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
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
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 +7 more
doaj +1 more source

