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Pathology General d4a6194c

Which is not a feature of paroxysmal nocturnal hemoglobinuria -

A
Increased LAP score
B
Thrombosis
C
Thrombocytopenia
D
Hemolysis
High-Yield Explanation
Clinical features of PNH A. Intravascular hemolysis The main feature of PNI-I is increased intravascular hemolysis that results in Hemoglobinemia            Hemoglobinuria               Hemosiderinuria Increased urine urobilinogen   Decreased serum haptoglobin  Increased serum LDH           Increased serum bilirubin The hemolysis is paroxysmal and usually occurs in the night because during sleep the pH of blood gets slightly reduced and acidic medium leads to activation of the complement. B. Thrombosis In PNH there is episodic thrombosis due to the absence of CD-59 on platelets, this results in the externalization of phosphatidylserine, a site for prothrombinase complexes and thus increase the propensity for thrombosis. Intrabdominal veins are the most common sites of thrombosis that may result in Budd Chiari syndrome due to hepatic vein thrombosis. C. Other features Thrombocytopenia Granulocytopenia Decreased LAP score Normoblastic hyperplasia of bone marrow PNH patients are also at increased risk for developing acute myelogenous leukemia and aplastic anemia.

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