Use to quantify or monitor paroxysmal nocturnal hemoglobinuria clone size. Preferred initial diagnostic test is Paroxysmal Nocturnal Hemoglobinuria (PNH), High Sensitivity, RBC and WBC (2005006).
Quantitative Flow Cytometry
New York State Clients: Testing is only approved for the Paroxysmal Nocturnal Hemoglobinuria Panel (ARUP test code 2005006) on whole blood specimens.
Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin).
Transport 4 mL whole blood. (Min: 4 mL)
Bone marrow. Clotted or hemolyzed specimens.
Specimens must be analyzed within stability times provided.
Ambient: 24 hours; Refrigerated: 72 hours; Frozen: Unacceptable
This test was developed according to published guidelines (Cytometry B Clin. Cytom. 2010; 78:211) and as updated in 2014 (Cytometry B Clin. Cytom. 2014; 86:44). WBC analysis is the most accurate measurement of the PNH clone size and uses FLAER and CD157 as GPI-linked markers with CD15 (PMNs) and CD64 (monocytes) as lineage-specific markers. The lower limits of detection are 0.005 percent for PMNs and 0.020 percent for monocytes.
For initial diagnosis of PNH, order High Sensitivity RBC and WBC Panel (ARUP test code 2005006). The presence of a subclinical PNH population in myelodysplastic bone marrow disorders, such as aplastic anemia or refractory anemia, may correlate with a positive immunotherapeutic response (Blood 2006; 107, 1308-1314).
For delineation of RBC Types II and III populations when the RBC clone size is greater than 1 percent, order PNH, High Sensitivity, RBC (ARUP test code 2004366).
Laboratory Developed Test (LDT)
|Component Test Code*||Component Chart Name||LOINC|
|2005004||% PNH Monocytes||60554-3|
|2005005||% PNH PMN||53831-4|
- (CD15, CD64, CD157, FLAER)
- Eculizumab treatment monitoring assay