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Seminars in Immunology, 2022
The treatment of paroxysmal nocturnal hemoglobinuria (PNH) was revolutionized by the introduction of the anti-C5 agent eculizumab, which resulted in sustained control of intravascular hemolysis, leading to transfusion avoidance and hemoglobin ...
A. Risitano +3 more
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The treatment of paroxysmal nocturnal hemoglobinuria (PNH) was revolutionized by the introduction of the anti-C5 agent eculizumab, which resulted in sustained control of intravascular hemolysis, leading to transfusion avoidance and hemoglobin ...
A. Risitano +3 more
semanticscholar +1 more source
Factor B inhibitor iptacopan for the treatment of paroxysmal nocturnal hemoglobinuria.
Blood reviewsParoxysmal nocturnal hemoglobinuria (PNH) is a rare, clonal, complement-mediated hemolytic anemia with a variety of manifestations. Currently, the methods for treating PNH include anti-C5 treatments (eculizumab and ravulizumab) and pegcetacoplan (a ...
Bo Xu +4 more
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Paroxysmal Nocturnal Hemoglobinuria
Mayo Clinic ProceedingsParoxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematologic disorder characterized by intravascular destruction of red blood cells by the complement system. This intravascular hemolysis can lead to a constellation of symptoms, including anemia, fatigue, shortness of breath, smooth muscle dystonia, and dark urine that is classically
Richard C. Godby, Surbhi Shah
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Pregnancy and Paroxysmal Nocturnal Hemoglobinuria
Archives of Internal Medicine, 1988Our study concerns eight pregnancies, six of which were successful, in four patients with paroxysmal nocturnal hemoglobinuria (PNH). Several complications of PNH during pregnancy were prevented: chronic anemia, folate and iron deficiency, and deep-vein thrombosis.
P, Solal-Céligny +8 more
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Paroxysmal Nocturnal Hemoglobinuria in Children
Pediatric Drugs, 2007Paroxysmal nocturnal hemoglobinuria (PNH), an acquired hematologic disorder characterized by intravascular hemolysis, nocturnal hemoglobinuria, thrombotic events, serious infections, and bone marrow failure, is very rare in children. PNH is caused by a somatic mutation of the phosphatidylinositol glycan (GPI) complementation class A (PIGA) gene ...
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Pregnancy and paroxysmal nocturnal hemoglobinuria
European Journal of Obstetrics & Gynecology and Reproductive Biology, 1994A patient is described who developed symptoms of paroxysmal nocturnal hemoglobinuria (PNH) in her first pregnancy. This was uneventful except for a spontaneous preterm delivery. The second pregnancy was complicated by severe anemia and a hemolytic crisis with Budd-Chiari syndrome at 31 weeks' amenorrhoea.
Bais, J. +3 more
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Paroxysmal Nocturnal Hemoglobinuria
New England Journal of Medicine, 1961PAROXYSMAL nocturnal hemoglobinuria is a disease with a fascinating diversity of challenges and implications. These have prompted numerous clinical and basic investigations. The clinical features are often bizarre and may be misleading, even with the most typical presentation of abdominal pain, fatigue, weakness, anemia and dark urine and with venous ...
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Paroxysmal Nocturnal Hemoglobinuria
1992Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of the hematopoietic stem cell (Hartmann and Arnold 1977) resulting in the production of blood cells which are defective in that they lack or are markedly deficient in glycan-phosphatidylinositol (GPI)-linked surface proteins (Rosse 1990a).
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Paroxysmal Nocturnal Hemoglobinuria Revisited
New England Journal of Medicine, 1983Immunologic systems are now known to have a major role in the pathophysiology of many human disease processes. One such system is serum complement.
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Paroxysmal nocturnal hemoglobinuria
Medicina clinica, 2009Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic disorder characterized by the existence of somatic mutations in the PIG-A (phosphatidylinositolglycan complementation class A) gene, which encodes for a protein involved in the biosynthesis of the glycosyl phosphatidylinositol (GPI) molecule that serves as an anchor for many ...
Pilar M, Hernández-Campo +2 more
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