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Pregnancy and paroxysmal nocturnal hemoglobinuria

European Journal of Obstetrics & Gynecology and Reproductive Biology, 1994
A 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., Pel, M., von dem Borne, A., van der Lelie, H.   +3 more
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Pegcetacoplan for paroxysmal nocturnal hemoglobinuria

Blood, 2022
AbstractApproximately a third of patients with paroxysmal nocturnal hemoglobinuria (PNH) remain transfusion dependent or have symptomatic anemia despite treatment with a C5 inhibitor. Pegcetacoplan inhibits complement proximally at the level of C3 and is highly effective in treating persistent anemia resulting from C3-mediated extravascular hemolysis ...
Gloria F. Gerber, Robert A. Brodsky
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Paroxysmal nocturnal hemoglobinuria

Hématologie, 2013
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells. PNH is related to a somatic mutation in the phosphatidylinositol glycan class A (PIG-A), X-linked gene, responsible for a deficiency in glycosylphosphatidylinositol-anchored proteins (GPI-AP).
R, Peffault de Latour, Z, Amoura, G, Socié   +2 more
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The syndrome of paroxysmal nocturnal hemoglobinuria

The American Journal of Medicine, 1964
Abstract The case histories and investigations are presented of four patients with paroxysmal nocturnal hemoglobinuria who satisfy the diagnostic criteria of abnormal erythrocyte acid hemolysis, positive intravascular hemolysis and low erythrocyte acetylcholinesterase. The clinical presentations of these patients ranged from a young man with a classic
R W, BEAL, H, KRONENBERG, B G, FIRKIN
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Paroxysmal Nocturnal Hemoglobinuria

Journal of Computer Assisted Tomography, 1993
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disorder, characterized by hemosiderin deposition in the proximal renal tubules.We examined six cases of PNH with MRI including gradient echo imaging.On T2- and T2*-weighted imaging a characteristic hypointense pattern was noted in five of six patients with PNH.
Y O, Tanaka, I, Anno, Y, Itai, T, Abe
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Paroxysmal Nocturnal Hemoglobinuria

Mayo Clinic Proceedings
Paroxysmal 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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Paroxysmal nocturnal hemoglobinuria in pregnancy

Acta Obstetricia et Gynecologica Scandinavica, 2003
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)‐anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria.
Line, Bjørge, Peter, Ernst, Kjell O, Haram   +2 more
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Thrombosis in paroxysmal nocturnal hemoglobinuria

Blood, 2013
Abstract The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the ...
Anita, Hill, Richard J, Kelly, Peter, Hillmen   +2 more
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Paroxysmal Nocturnal Hemoglobinuria

Complement and Inflammation, 2017
Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disease characterized by an increased sensitivity of erythrocytes to the hemolytic action of complement. Two membrane proteins, the decay-accelerating factor and the C8-binding protein, which protect normal erythrocytes from the hemolytic action of complement, are deficient on the abnormal blood ...
Jose A. Halperin, Anne Nicholson-Weller
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Pregnancy and Paroxysmal Nocturnal Hemoglobinuria

Archives of Internal Medicine, 1988
Our 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, G, Tertian, H, Fernandez, J C, Pons, T, Lambert, Y, Najean, J P, Clauvel, E, Papiernik, G, Tchernia   +8 more
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