P.N.H is associated with all of the following conditions,except:
Correct Answer: Increased LAP scores
Description: PNH is not associated with increased LAP scores. In PNH, there is loss of hemoglobin and iron in urine - the negative iron deficit leading to iron deficiency anemia. The hypercoagulable state in PNH leads to Budd Chiari syndrome and this disorder, being an acquired stem cell defect can lead to aplastic anemia. The clinical syndrome can present with 3 types of symptoms including: (1) an acquired intracorpuscular hemolytic anemia due to the abnormal susceptibility of the RBC membrane to the hemolytic activity of complement; (2) thrombosis in large vessels, such as hepatic, abdominal, cerebral, and subdermal veins; and (3) a deficiency in hematopoiesis that may be mild or severe, such as pancytopenia in an aplastic anemia state. Flow cytometry is used to detect CD59 (MIRL), a glycoprotein, and CD55 (DAF) in regulation of complement action. Absence or reduced expression of both CD59 and CD55 on PNH RBCs is diagnostic. The Ham test (acidified serum lysis) establishes the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), demonstrating a characteristic abnormality of PNH red blood cells by acidified fresh normal serum. Heated serum at 56degC inactivates complement and prevents hemolysis in PNH cells.
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