Ordering Recommendation

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).

Mnemonic
PNH WBC
Methodology

Quantitative Flow Cytometry

Performed

Sun-Sat

Reported

1-3 days

New York DOH Approval Status
Specimens from New York clients will be sent out to a New York DOH approved laboratory, if possible.
Specimen Required
Patient Preparation

New York State Clients: Testing is only approved for the Paroxysmal Nocturnal Hemoglobinuria Panel (ARUP test code 2005006) on whole blood specimens.

Collect

Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin).

Specimen Preparation

Transport 4 mL whole blood. (Min: 4 mL)

Storage/Transport Temperature

Refrigerated.

Unacceptable Conditions

Bone marrow. Clotted or hemolyzed specimens.

Remarks

Specimens must be analyzed within stability times provided.

Stability

Ambient: 24 hours; Refrigerated: 72 hours; Frozen: Unacceptable

Reference Interval

By report

Interpretive Data

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).

Compliance Category

Laboratory Developed Test (LDT)

Note
Hotline History
N/A
CPT Codes

86356 x5

Components
Component Test Code* Component Chart Name LOINC
2005004 % PNH Monocytes 60554-3
2005005 % PNH PMN 53831-4
* 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.
Aliases
  • (CD15, CD64, CD157, FLAER)
  • Eculizumab treatment monitoring assay
Paroxysmal Nocturnal Hemoglobinuria, High Sensitivity, WBC