Which is not a feature of paroxysmal nocturnal hemoglobinuria –
Correct Answer: Increased LAP score
Description: 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.
Category:
Pathology
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