DryFlowEx PNH High-Sensitivity Assay Kit (CE IVD)

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder resulting as a consequence of the non-malignant clonal expansion of cells with somatic mutation of Phosphatidylinositol Glycan Anchor Biosynthesis Class A (PIGA) gene. Mutations of the PIGA gene result in the inability to express glycosylphosphatidyl-inositol (GPI)-anchored cell surface proteins. The device is intended to detect GPI-deficient neutrophil granulocytes and monocytes, together with complete (Type III) and partial (Type II) GPI-deficient erythrocytes for evaluation of the PNH clone size. Furthermore, the device detects GPI-deficient reticulocytes (immature erythrocytes) in patients receiving blood transfusions when PNH erythrocytes are difficult to delineate.

Intended Purpose:
DryFlowEx PNH High-Sensitivity Assay Kit is intended for high sensitivity detection and enumeration of glycosyl-phosphatidyl-inositol (GPI)-deficient cells in human whole blood by flow cytometry.

What is detected and/or measured:
The device DryFlowEx PNH High-Sensitivity Assay Kit detects and enumerates glycosyl-phosphatidyl-inositol (GPI)-deficient cells (PNH clones) as a percentage of:
• CD59 dim or CD59- cells from all erythrocytes (CD235a+)
• CD59 dim or CD59- cells from all reticulocytes (CD235a+CD71+)
• CD14-, CD157- and Proaerolysin- cells from all monocytes (CD45+CD64+)
• CD24-, CD157- and Proaerolysin- cells from all neutrophil granulocytes (CD45+CD15+)

Device function:
The device is intended for diagnosis and monitoring of patients suffering, or suspected of suffering Paroxysmal Nocturnal Hemoglobinuria (PNH) and related disorders.

Table 1: Description of the PNH WBC 7-color active ingredients
 

Antigen	Fluorochrome	Clone	Isotype
GPI anchor (Proaerolysin)	Alexa Fluor®488	N/A	N/A
CD157	PE	SY11B5	IgG1
CD45	PerCP-Cy™5.5	2D1	IgG1
CD64	PE-Cy™7	10.1	IgG1
CD24	APC	SN3	IgG1
CD14	APC-Cy™7	MEM-15	IgG1
CD15	Pacific Blue™	MEM-158	IgM

  
Table 2: Description of the PNH RBC 3-color active ingredients

Antigen	Fluorochrome	Clone	Isotype
CD235a	FITC	JC159	IgG1
CD59	PE	MEM-43	IgG2a
CD71	APC	MEM-75	IgG1

 

Ordering information

Cat. No.:	Title:	Regulatory status:	Quantity:
ED7750	DryFlowEx PNH High-Sensitivity Assay Kit	CE IVD	25 tests

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Further reading:
Sutherland DR, Kuek N, Davidson J, Barth D, Chang H, Yeo E, Bamford S, Chin-Yee I, Keeney M: Diagnosing PNH with FLAER and multiparameter flow cytometry. Cytometry B Clin Cytom. 2007 May;72(3):167-77.
 
Borowitz MJ, Craig FE, Digiuseppe JA, Illingworth AJ, Rosse W, Sutherland DR, Wittwer CT, Richards SJ: Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry. Cytometry B Clin Cytom. 2010 Jul;78(4):211-30.
 
Sachdeva MU, Varma N, Chandra D, Bose P, Malhotra P, Varma S: Multiparameter FLAER-based flow cytometry for screening of paroxysmal nocturnal hemoglobinuria enhances detection rates in patients with aplastic anemia. Ann Hematol. 2015 May;94(5):721-8. doi: 10.1007/s00277-014-2267.
 
Marinov I, Illingworth AJ, Benko M, Sutherland DR. Performance Characteristics of a Non-Fluorescent Aerolysin-Based Paroxysmal Nocturnal Hemoglobinuria (PNH) Assay for Simultaneous Evaluation of PNH Neutrophils and PNH Monocytes by Flow Cytometry, Following Published PNH Guidelines. Cytometry B Clin Cytom. 2018 Mar;94(2):257-263. doi: 10.1002/cyto.b.21389. Epub 2016 Jul 6. PMID: 27294344.

Sutherland DR, Ortiz F, Quest G, Illingworth A, Benko M, Nayyar R, Marinov I. High-sensitivity 5-, 6-, and 7-color PNH WBC assays for both Canto II and Navios platforms. Cytometry B Clin Cytom. 2018 Jul;94(4):637-651. doi: 10.1002/cyto.b.21626. Epub 2018 Mar 5. PMID: 29381839.
 
Brando B, Gatti A, Preijers F: Flow cytometric diagnosis of paroxysmal nocturnal hemoglobinuria: Pearls and pitfalls - a critical review article. EJIFCC. 2019 Nov 25;30(4):355-370.
 
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 May;41 Suppl 1:73-81.

Sutherland DR, Richards SJ, Ortiz F, Nayyar R, Benko M, Marinov I, Illingworth A. CD71 improves delineation of PNH type III, PNH type II, and normal immature RBCS in patients with paroxysmal nocturnal hemoglobinuria. Cytometry B Clin Cytom. 2020 Mar;98(2):179-192. doi: 10.1002/cyto.b.21853. Epub 2019 Nov 8. PMID: 31705743.