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What is Which of the following is a cause of intravascular hemolysis and explain in detail?
PNH * Paroxysmal nocturnal hemoglobinura (PNH ) is an acquired intrinsic defect in RBC membrane * Its due to mutation in PIGA gene (phosphatidyl inositol glycan complementation group A) PNH red cell are deficient in :- * CD55 or decay accelerating factor * CD 59 or membrane inhibitor of reactive lysis * C8 binding protein * Characterised by pancytopenia, hemolysis and thrombosis * Diagnosed by FLOWCYTOMETRIC ANALYSIS * Thrombosis is the leading cause of disease related death Ref:- Robbins Basic Pathology 10th Edition; Pg num:- 642,643