Rev Assoc Med Bras ; 56 2 : Brodsky R. Narrative review: Paroxysmal nocturnal hemoglobinuria: the physiology of complement-related hemolytic anemia. Multicenter phase 3 study of the complement inhibitor eculizumab for thetreatment of patients with paroxysmal nocturnal hemoglobinuria. Predictors of hemoglobin response to eculizumab therapy in paroxysmal nocturnal hemoglobinuria. Eur J Haematol.

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Salamanca: Ediciones Universal; Paroxysmal Nocturnal Hemoglobinuria. Present status and future prospects. West J Med ; Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria: certain inmunological aspects of the hemolytic mechanism with special reference to serum complement.

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The sensitivity of PNH red blood cells to lysis complement and specific antibody. The population of cells in paroxysmal nocturnal haemoglobinuria of intermediate sensitivity to complement lysis: significance and mechanism of increased immune lysis. Br J haematol ; Complement lysis of human erythrocytes. Differing susceptibility of two types of paroxysmal nocturnal hemoglobinuria cells to C5b The sugar-water test for paroxysmal nocturnal hemoglobinuria.

A potencial screening test for PNH. N Engl J Med ; Sangre ; Deficiency of an erythrocyte memebrane protein with complement regulatory activity in paroxysmal nocturnal hemoglobinuria.

Affected erythrocytes of patients of patients with Paroxysmal Nocturnal Hemoglobinuria are deficient in the complement regulatory protein decay accelerating factor. Decay-accelarating factor is present on paroxysmal nocturnal hemoglobinuria erythroid progenitors and lost during erythropoiesis in vitro. J Exp Med ; Deficiency of the hologous restriction factor in paroxysmal nocturnal hemoglobinuria. Paroxysmal nocturnal hemoglobinuria. Enhanced estimulation of platelets by the terminal complement components is related to the lack of C8bp in the membrane.

J Immunol ; Isolation and characterization of a membrane protein from normal human erythrocytes that inhibits reactive lysis of the erythrocytes of paroxysmal nocturnal haemoglobinuria.

J Clin Invest ; Rev Clin Esp ; Defective glycosylphosphatidylinositol anchor syntesis in paroxysmal nocturnal hemoglobinuria granulocytes. Synthesis of mannosylglucosaminylinositol phosphoplipids in normal but not paroxysmal nocturnal hemoglobinuria cells. Deficient biosynthesis of -acetyl-glucosaminyl-phosphatidylinositol, the first intermediate of glycosyl phosphatidylinositol anchor biosynthesis, in cell lines established from patients with paroxysmal nocturnal hemoglobinuria.

Science ; Cell ; Somatics mutations of the PIG-A gene found in Japanese patients with paroxysmal nocturnal hemoglobinuria. Somatic mutations and cellular selection in paroxysmal nocturnal haemoglobinuria. Lancet ; Blood ; Chromosomic findings in patients with paroxysmal nocturnal hemoglobinuria. Int J Hematol ; Emergence of CD52,phosphatidylinositolglycan-anchor-deficient T lymphocytes after in vivo application of Campath-1H for refractory Bcell non Hodgkin lymphoma.

Rev Invest Clin ; Paroxysmal nocturnal haemoglobinuria in Thailand with special reference to an association with aplasic anaemia. Br J Haematol ; Paroxysmal Nocturnal Hemoglobinuria: The mexican experience. Rev Invest Clin ; 49 supl : Prognostic features of paroxysmal nocturnal haemoglobinuria in Japan. Acta Haematol Jpn ; Bol Med Hosp Infant Mex ; Sangre ; Expression of phosphatidylinositol anchored membrane proteins in paroxysmal nocturnal haemoglobinuria after bone marrow transplantation.

Bone Marrow Transplant ;

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Paroxysmal nocturnal hemoglobinuria PNH is a rare, acquired, [1] life-threatening disease of the blood characterized by destruction of red blood cells by the complement system , a part of the body's innate immune system. This destructive process occurs due to the presence of defective surface protein DAF on the red blood cell , which normally functions to inhibit such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system , the red blood cell destruction hemolysis is considered an intravascular hemolytic anemia. Other key features of the disease, such as the high incidence of blood clot formation , are incompletely understood.


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Paroxysmal nocturnal hemoglobinuria


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