Paroxysmal Nocturnal Hemoglobinuria, PI-Linked Antigen, Blood
Use
This test is useful for screening and confirming the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) and for monitoring patients with PNH. PNH is an acquired hematologic disorder characterized by nocturnal hemoglobinuria, chronic hemolytic anemia, thrombosis, pancytopenia, and acute or chronic myeloid malignancies in some patients. It is a stem cell disorder affecting various blood cell lines, resulting in a deficiency of GPI-linked proteins. Variants in the PIGA gene are commonly identified in PNH patients, confirming the biological defect. Flow cytometry assays detect GPI-linked protein absence in blood cells, providing a more reliable diagnosis by avoiding the issues of RBC-based diagnostic methods.
Special Instructions
Not provided.
Limitations
This assay replaces the sugar water test and the Ham test for evaluating PNH, providing more reliability in detecting all Ham-positive PNH cases and some Ham-negative cases. Recent hemolytic episodes or transfusions may interfere with RBC-based diagnosis, leading to false negatives. While PNH affects stem cells, the percentage of affected cells can vary between lineages due to hemolysis and/or transfusion. It is important to note that recent transfusions can decrease the sensitivity of the test and interfere with accuracy.
Methodology
Cell-based / Cytometry (Flow Cytometry)
Biomarkers
CD55
ProteinCD59
ProteinFLAER
Protein
LOINC Codes
- 90735-2 - PNH GPI-Lkd WBC + RBC Ag Pnl Bld
- 90739-4 - PNH GPI-Linked WBC + RBC Ag Bld-Imp
- 90738-6 - RBC CD59 complete loss NFr
- 90737-8 - CD24-FLAER- cells/CD15 NFr Bld
- 90736-0 - CD14-FLAER- cells/CD33 NFr Bld
Result Turnaround Time
1-3 days
Related Documents
For more information, please review the documents below
Specimen
Whole Blood
Volume
2.6 mL
Minimum Volume
1 mL
Container
Yellow top (ACD solution A or B) preferred; Lavender top (EDTA) acceptable
Collection Instructions
Send whole blood specimen in original tube. Do not aliquot.
Storage Instructions
Specimen must arrive within 72 hours of collection.
Causes for Rejection
Gross hemolysis, Fully clotted
Stability Requirements
| Temperature | Period |
|---|---|
| Room Temperature | 72 hours |
| Refrigerated | 72 hours |
