This video is all about a rare clonal hematopoetic disorder called paroxysmal nocturnal hemoglobinuria abbreviated as PNH. PNH is because of the mutation in PIG A gene which codes for GPI (glycophosphotidylinositol). GPI is needed for anchoring DAF and CD59 on red cell membrane. In the absence of DAF and CD59 the red cells will be hemolysed by the compliment system. Complimented mediated hemolysis in paroxysmal nocturnal hemoglobinuria, PNH, leads to complement mediated intravascular hemolysis giving rise to hemoglobinuria, jaundice and anemia. PNH is diagnosed using sucrose hemolysis test or sugar water test, Hams test and flow cytometry.
Understanding cellular metabolic process will help us to understand the disease process when some metabolic events go wrong!! With this blog I would like to share my medical biochemistry knowledge with you!!!
Sunday, March 19, 2017
Paraxysmal Nocturnal Hemoglobinuria (PNH)
This video is all about a rare clonal hematopoetic disorder called paroxysmal nocturnal hemoglobinuria abbreviated as PNH. PNH is because of the mutation in PIG A gene which codes for GPI (glycophosphotidylinositol). GPI is needed for anchoring DAF and CD59 on red cell membrane. In the absence of DAF and CD59 the red cells will be hemolysed by the compliment system. Complimented mediated hemolysis in paroxysmal nocturnal hemoglobinuria, PNH, leads to complement mediated intravascular hemolysis giving rise to hemoglobinuria, jaundice and anemia. PNH is diagnosed using sucrose hemolysis test or sugar water test, Hams test and flow cytometry.
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