Laboratory Medicine Program
Paroxysmal Nocturnal Hemoglobinuria (PNH)
(CD59, FLAER, Flow Cytometry)
Clinical Decription:
Patients with paroxysmal nocturnal hemoglobinuria cannot synthesize GPI due to an acquired somatic mutation in the PIGA gene in hematopoetic stem cells. The purpose of this test is to detect GPI-deficient red and white blood cells.
Total RBCs are analyzed for decreased expression of CD59. In case of a PNH clone 10% or higher, the size of the PNH clone is determined on reticulocytes identified by the antibody CD71. In addition, RBCs are also evaluated for the binding of complement factor 3d (C3d). RBC opsonized with C3d are prone to extravascular hemolysis which occurs in patients with breakthrough hemolysis.
Monocytes and granulocytes are analyzed for decreased expression of CD157 and reduced or absent binding of fluorescent aerolysin (FLAER).
Method: Flow Cytometry; BD FACS Lyrics
Component Tests Used: n/a
Reference Ranges Used:
Reference ranges for this test are not available online. However, they are included in all test results. For more information, please call us.
Specimen Type: Peripheral Blood
Collected In: EDTA
Volume: 3mL mL (minimum: 3mL mL)
Shipping: Room temperature
Special Instructions: Samples received greater than 48 hours from the time of collection may yield suboptimal results; requests and collections for testing should be made in alignment with stability and Flow Cytometry laboratory operating hours (business hours).
Testing Schedule(s): Monday - Thursday (0900-1700), Friday (0900-1200).
Turnaround Time: 7 days
For more information, call 416.340.5227 or 1.866.865.5227