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Rare and acquired
Unknown etiology; comes from a somatic mutation of affecting a pluripotent stem cell
RBCs, granulocytes, and platelets are very sensitive to complement-mediated lysis due to a lack of 3 normal membrane proteins: decay accelerating factor (CD55), membrane inhibitor of reactive lysis (CD59) and a C8-binding protein; hemolysis may worsen at night
Chronic hemosiderinuria may lead to iron deficiency
Nonlytic complement interactions with granulocyte and platelet membranes predispose to infections and thrombosis (especially in the hepatic, portal, and cerebral veins)
There is an increased risk for aplastic anemia and acute leukemia because this is a stem cell d/o
Diagnosis can be suggested by a (+) sucrose hemolysis test and confirmed by performing an acidified serum test (Ham’s test) – flow cytometry of WBC and RBC levels of CD55 and CD59 is currently the gold standard
Median survival is 10 years
Category: Medical Subject Notes , Pathology Notes
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