Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin).
Only collect Monday through Friday, must be IN the Sendout Lab by 1200 on Friday for shipment. Do not draw on weekends or holidays.
Specimen Preparation
Transport 4 mL whole blood. (Min: 4 mL)
Unacceptable Conditions
Clotted or hemolyzed specimens.
Stability (from collection to initiation)
Ambient: 24 hours; Refrigerated: 72 hours; Frozen: Unacceptable New York State Clients: Ambient: 24 hours; Refrigerated: 48 hours. Frozen: Unacceptable
Performed
Sun-Sat
Remarks
Specimens must be analyzed within stability times provided.
Notes
If >1% PNH RBCs are detected, then PNH RBC TYPE reflex will be added at no additional charge.
Ordering Recommendations
Preferred test for initial diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) and quantification of PNH clones.
Performed
Sun-Sat
Methodology
Quantitative Flow Cytometry
Synonyms
CD15, CD33, CD14, CD24, FLAER, CD59, Glycophorin A
PI-Linked Antigens, RBCs, Monocytes and Granulocytes
CD157, FLAER, CD59, Glycophorin A
PI-Linked, eculizumab monitoring
ARUP Test Code
2005006
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 2018 (Cytometry B Clin. Cytom. 2018; 94B:49). The test includes high-sensitivity WBC and RBC analysis with a lower limit of quantification of 0.02 percent for PNH RBCs and PMNs (based on 250,000 cells analyzed) and 0.5 percent for PNH monocytes (based on 10,000 cells analyzed). The lower limit of detection for PNH RBCs and PMNs is 0.008 percent and for PNH monocytes 0.2 percent. For severely pancytopenic patients, the WBC assay sensitivity will be much lower.
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).
Patient Retesting Recommendations: The frequency of testing is dictated by clinical and hematologic parameters; repeat testing is indicated upon any significant change in clinical or laboratory parameters and is suggested at least annually for routine monitoring. In the setting of aplastic anemia, international guidelines recommend screening for PNH at diagnosis, and every 3 to 6 months initially, reducing the frequency of testing if the proportion of GPI-deficient cells has remained stable over an initial two-year period (Int J Lab Hematol 2019;41 Suppl 1:73-81).
This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the U.S. Food and Drug Administration. This test was performed in a CLIA-certified laboratory and is intended for clinical purposes.
Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin).
Only collect Monday through Friday, must be IN the Sendout Lab by 1200 on Friday for shipment. Do not draw on weekends or holidays.
Specimen Preparation
Transport 4 mL whole blood. (Min: 4 mL)
Unacceptable Conditions
Clotted or hemolyzed specimens.
Stability (from collection to initiation)
Ambient: 24 hours; Refrigerated: 72 hours; Frozen: Unacceptable New York State Clients: Ambient: 24 hours; Refrigerated: 48 hours. Frozen: Unacceptable
Performed
Sun-Sat
Remarks
Specimens must be analyzed within stability times provided.
Notes
If >1% PNH RBCs are detected, then PNH RBC TYPE reflex will be added at no additional charge.
Ordering
Ordering Recommendations
Preferred test for initial diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) and quantification of PNH clones.
Performed
Sun-Sat
Methodology
Quantitative Flow Cytometry
Synonyms
CD15, CD33, CD14, CD24, FLAER, CD59, Glycophorin A
PI-Linked Antigens, RBCs, Monocytes and Granulocytes
CD157, FLAER, CD59, Glycophorin A
PI-Linked, eculizumab monitoring
ARUP Test Code
2005006
Result Interpretation
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 2018 (Cytometry B Clin. Cytom. 2018; 94B:49). The test includes high-sensitivity WBC and RBC analysis with a lower limit of quantification of 0.02 percent for PNH RBCs and PMNs (based on 250,000 cells analyzed) and 0.5 percent for PNH monocytes (based on 10,000 cells analyzed). The lower limit of detection for PNH RBCs and PMNs is 0.008 percent and for PNH monocytes 0.2 percent. For severely pancytopenic patients, the WBC assay sensitivity will be much lower.
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).
Patient Retesting Recommendations: The frequency of testing is dictated by clinical and hematologic parameters; repeat testing is indicated upon any significant change in clinical or laboratory parameters and is suggested at least annually for routine monitoring. In the setting of aplastic anemia, international guidelines recommend screening for PNH at diagnosis, and every 3 to 6 months initially, reducing the frequency of testing if the proportion of GPI-deficient cells has remained stable over an initial two-year period (Int J Lab Hematol 2019;41 Suppl 1:73-81).
This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the U.S. Food and Drug Administration. This test was performed in a CLIA-certified laboratory and is intended for clinical purposes.
