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Paroxysmal Cold Hemoglobinuria
Hematology/Oncology Clinics of North America, 2015Paroxysmal cold hemoglobinuria is a rare cause of autoimmune hemolytic anemia predominantly seen as an acute form in young children after viral illnesses and in a chronic form in some hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on ...
Satish, Shanbhag, Jerry, Spivak
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Pegcetacoplan for paroxysmal nocturnal hemoglobinuria
Blood, 2022AbstractApproximately 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, 2013Paroxysmal 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 +2 more
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PAROXYSMAL COLD HEMOGLOBINURIAS
Archives of Internal Medicine, 1948DRESSLER 1 is credited with having first reported observations on a case of "paroxysmal cold hemoglobinuria," a symptom complex now known to have two separate and distinct etiologic bases. The first and more generally recognized type is syphilitic paroxysmal cold hemoglobinuria, a disease seen in a small percentage of persons with syphilis and caused ...
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Paroxysmal Cold Hemoglobinuria
CRC Critical Reviews in Clinical Laboratory Sciences, 1978The clinical and hematological features of a rare autoimmune disorder, PCH, are reviewed. Based on the case reports of 24 patients suffering from this disease, the presence of cold hemolysins in the sera of these patients as the main cause for the red cell destruction following exposure to cold is further discussed.
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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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