ARUP's Laboratory Test Directory

Paroxysmal Nocturnal Hemoglobinuria Profile, High Resolution - RBC : 0095864

Mnemonic: PNH

Methodology: Flow Cytometry
Performed: Sun-Sat
Reported: 1-4 days
Specimen Required: Collect:  One 5 mL green (sodium or lithium heparin), lavender (EDTA), or pink (K2EDTA).

Transport:  5 mL whole blood at 2-8°C. (Min: 0.5 mL)

Remarks:  New York Clients are required to order both the PNH (0095864) and the PNH-WBC (096666) tests.  NY samples must be <24 hours old.

Stability:  Ambient: 4 days; Refrigerated: 4 days; Frozen: Unacceptable
Reference Interval:
 
Test Number Components Reference Interval
 % Normal RBCs Normal: 99.995-100.000%
 % PNH Cells Negative: 0.000-0.005%

Interpretive Data: The High Resolution PNH-RBC panel detects the absence of phosphatidylinositol-linked membrane proteins CD55 (DAF-decay accelerating factor) and CD59 (MIRL-membrane inhibitor of reactive lysis) on erythrocytes. The erythrocytes are identified with glycophorin staining. All normal RBCs (99.995-100.000%) express CD55 and CD59 and are reported as % Normal RBCs. The expression of CD55 and CD59 is decreased or absent in a subpopulation of RBCs in patients with paroxysmal nocturnal hemoglobinuria. The estimated percentage of RBCs with decreased expression is reported as % PNH cells.  Since 'diagnosis of PNH disease requires a deficiency of at least two GPI-linked antigens in two different cell lineages'*, this test may be complemented with a PNH-WBC test (test code 096666) if clinically indicated.  Please note that recent RBC transfusions may artifactually decrease the percentage of PNH cells. *Cytometry 42:223-33 (2000)

The detection of a very low percentage of PNH cells (0.006-1.000%) may be found in patients with aplastic anemia, myelodysplasia, refractory anemia, refractory anemia with excess blasts, and myelofibrosis. The presence of PNH cells in these patients may correlate with a better prognosis. Blood 102:1211-1218 (2003)

Please refer to Statement A in the Compliance Statements section in the front of the Laboratory Test Directory.
Note: This panel tests for the phosphatidylinositol-linked membrane proteins CD55 (DAF-decay accelerating factor) and CD59 (MIRL-membrane inhibitor of reactive lysis) on erythrocytes. The percentage of RBCs with normal expression of CD55 and CD59 is decreased in paroxysmal nocturnal hemoglobinuria (PNH).
CPT Code(s): 88184 First marker; 88185 x2 Each additional marker
 
 

 

 

 
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