DR KUNAL MAHAJAN: few things on PNH....important topic aiims/allindia

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DR KUNAL MAHAJAN

mbbs(rpgmc tanda)

md med student(pgims rohtak)

Friday, April 9, 2010

few things on PNH....important topic aiims/allindia

PNH(paroxysmal nocturnal haemoglobinuria)...some important facts

pathogenesis-----this is due to mutation in PIGA gene(phosphatidylinositol glycosylated A gene) =>causing deficiency of GPI(glycosylated phosphatidyl inositol) which binds certain protective proteins to
cell membranes of RBCs,platelets and WBCs,these protective proteins PROTECT THE CELLS (RBC/WBC/PLATELETS) FROM COMPLEMENT MEDIATED LYSIS
these protective proteins are 1)CD 59(also k/a MIRL=membrane inhibitor of reactive lysis) ; 2) CD55(also k/a decay accelerating factor) ; 3)C8 binding protein,etc............out of these,CD59 is the most important

so PIGA mutation => GPI deficiency => deficiency of CD59,CD55,etc =>leading to complement mediated lysis of RBCs,platelets and WBCs

MCQs on PNH features
1)LAP score is decreased
2)pancytopenia with hyper/hypo/normocellular bone marrow is seen
3)gold standard for diagnosis-flow cytometry
4)other tests used for diagnosis--HAM oxidase test,,,,,,sucrose hemolysis test....but these are not reliable
5)ECULIZUMAB----monoclonal antibody against complement protein C5.....used in treatment of PNH