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HelpTest Code PLINK Paroxysmal Nocturnal Hemoglobinuria, PI-Linked Antigen, Blood
Reporting Name
PNH, PI-Linked AG, BUseful For
Screening for and confirming the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)
Monitoring patients with PNH
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Specimen Type
Whole bloodSpecimen Required
Specimen must arrive within 3 days of collection.
Container/Tube:
Preferred: Yellow top (ACD solution A or B)
Acceptable: Lavender top (EDTA)
Specimen Volume: 2.6 mL
Collection Instructions: Send whole blood specimen in original tube. Do not aliquot.
Specimen Minimum Volume
1 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Whole blood | Ambient (preferred) | 72 hours | |
| Refrigerated | 72 hours |
Reference Values
An interpretive report will be provided.
RED BLOOD CELLS:
PNH RBC-Partial Antigen loss: 0.00-0.99%
PNH RBC-Complete Antigen loss: 0.00-0.01%
PNH Granulocytes: 0.00-0.01%
PNH Monocytes: 0.00-0.05%
Day(s) Performed
Monday through Saturday
Test Classification
This test was developed using an analyte specific reagent. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
88184-Flow cytometry, RBC x 1
88184-Flow cytometry, WBC x 1
88185-Flow cytometry, additional marker (each), RBC x 1
88185-Flow cytometry, additional marker (each), WBC x 6
88188-Flow Cytometry Interpretation, 9-15 Markers x 1
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PLINK | PNH, PI-Linked AG, B | 90735-2 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| CK079 | Interpretation | 90739-4 |
| CK080 | PNH RBC-Partial Ag Loss | 33662-8 |
| CK081 | PNH RBC-Complete Ag Loss | 90738-6 |
| CK082 | PNH Granulocytes | 90737-8 |
| CK083 | PNH Monocytes | 90736-0 |
Interpretation
Individuals with paroxysmal nocturnal hemoglobinuria (PNH) have absent or decreased expression of all the glycosylphosphatidylinositol (GPI)-linked antigens and fluorescent aerolysin (FLAER) on peripheral blood cells derived from the PNH clone.
Recent data showed that small PNH clones can be detected in a relatively high percentage of cases of aplastic anemia and myelodysplastic syndrome. While the significance of this finding is still uncertain, it appears that these patients may benefit from immunosuppressive therapy.
This test incorporates a sophisticated technique of separating different cell populations using gating on antigen-positive cells, as well as the sensitivity to enable detection of small PNH clones. In addition, this test detects a partial loss of CD59 on type II red blood cells (RBC). Patients with large proportion of type II RBC are unlikely to show high levels of hemolysis, unlike patients with complete loss of GPI-linked proteins (predominantly type III cells). While PNH is a disorder of hematopoietic stem cells and all lineages are affected, the percentage of affected cells can differ between lineages, most commonly due to hemolysis and/or transfusion.
Individuals without PNH have normal expression of FLAER (neutrophils and monocytes) and normal expression of all GPI-linked antigens-CD14 (monocytes), CD16 (neutrophils and NK cells), CD24 (neutrophils), and CD59 (RBC).
Clinical Reference
1. Richards SJ, Hill A, Hillman P. Recent advances in the diagnosis, monitoring and management of patients with paroxysmal nocturnal hemoglobinuria. Cytometry B Clin Cytom. 2007;72(5):291-298
2. Sutherland DR, Illingworth A, Marinov I, et al. ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 2 - reagent selection and assay optimization for high-sensitivity testing. Cytometry B Clin Cytom. 2018;94(1):23-48. doi:10.1002/cyto.b.21610
3. Illingworth A, Marinov I, Sutherland DR, Wagner-Ballon O, DelVecchio L. ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 3 - data analysis, reporting and case studies. Cytometry B Clin Cytom. 2018;94(1):49-66. doi:10.1002/cyto.b.21609
4. Oldaker T, Whitby L, Saber M, Holden J, Wallace PK, Litwin V. ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 4 - assay validation and quality assurance. Cytometry B Clin Cytom. 2018;94(1):67-81. doi:10.1002/cyto.b.21615
5. Dezern AE, Borowitz MJ: ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 1 - clinical utility. Cytometry B Clin Cytom. 2018;94(1):16-22. doi:10.1002/cyto.b.21608
6. Illingworth AJ, Marinov I, Sutherland DR. Sensitive and accurate identification of PNH clones based on ICCS/ESCCA PNH Consensus Guidelines-A summary. Int J Lab Hematol. 2019;41 Suppl 1:73-81. doi:10.1111/ijlh.13011
7. Seth N, Mahajan V, Kedia S, Sutar A, Sehgal K. Utility of FLAER and CD157 in a five-color single-tube high sensitivity assay, for diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH)-A standalone flow cytometry laboratory experience. Int J Lab Hematol. 2021;43(2):259-265. doi:10.1111/ijlh.13366
8. Payne D, Johansson U, Bloxham D, et al. Inter-laboratory validation of a harmonized PNH flow cytometry assay. Cytometry B Clin Cytom. 2018;94(5):580-587. doi:10.1002/cyto.b.21726
9. Sutherland DR, Ortiz F, Quest G, et al. High-sensitivity 5-, 6-, and 7-color PNH WBC assays for both Canto II and Navios platforms. Cytometry B Clin Cytom. 2018;94(4):637-651. doi:10.1002/cyto.b.21626
Report Available
1 to 3 daysMethod Name
Immunophenotyping
Additional Tests
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| FCIMS | Flow Cytometry Interp, 9-15 Markers | No, (Bill Only) | Yes |
Forms
If not ordering electronically, complete, print, and send 1 of the following forms with the specimen: