Paroxysmal Nocturnal Hemoglobinuria (PNH), High Sensitivity, RBC and WBC
Ordering Recommendation

Preferred test for initial diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) and quantification of PNH clones.

Quantitative Flow Cytometry
1-3 days
New York DOH Approval Status
This test is New York DOH approved.
Submit With Order
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin). 
Specimen Preparation
Transport 4 mL whole blood. (Min: 4 mL) 
Storage/Transport Temperature
Unacceptable Conditions
Bone marrow. Clotted or hemolyzed specimens. 
Specimens must be analyzed within stability times provided. 
Ambient: 24 hours; Refrigerated: 72 hours; Frozen: Unacceptable
New York State Clients: Ambient: 24 hours; Refrigerated: 48 hours. Frozen: Unacceptable 
Reference Interval
By report
Interpretive Data
This test is preferred for the initial diagnosis of PNH, and 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). The test includes high-sensitivity WBC and RBC analysis with lower limits of detection of 0.005 percent for RBCs, 0.005 percent for PMNs, and 0.020 percent for monocytes.

WBC analysis is the most accurate measurement of the PNH clone size. FLAER and CD157 are used as GPI-linked markers; CD15 (PMNs) and CD64 (monocytes) are used as lineage-specific markers. RBC analysis quantifies Type II and Type III RBC clones when the percentage of PNH RBCs is greater than 1 percent. Glycophorin A (CD235a) is used to gate the RBC population, and CD59 is the GPI-linked antigen. Recent RBC transfusions may decrease the percentage of PNH cells measured in RBCs (Cytometry 2000; 42:223). 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).

Compliance Statement A: For laboratory developed tests using a manufacturer labeled ASR as the reagent providing the specificity of the assay. Analyte Specific Reagent. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, the FDA has determined that such clearance or approval is not necessary.

Hotline History
Component Test Code*Component Chart NameLOINC
2004367% PNH RBC33662-8
2005004% PNH Monocytes60554-3
2005005% PNH PMN53831-4
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.
  • CD15, CD33, CD14, CD24, FLAER, CD59, Glycophorin A
  • PI-Linked Antigens, RBCs, Monocytes and Granulocytes