Hematology > ID-PNH Test


(Id-n°: 50620)

(Id-n°: 45270)

Rapid test for the diagnosis and therapy monitoring of paroxysmal nocturnal hemogloginuria (PNH).

Reagent 1:
ID-PNH-Gel card with rabbit antiserum against mouse immunoglobulines.

Reagent 2:
Monoclonal antibodies directed against DAF and MIRL respectively, and negative control, ready-to-use, 1.4 mL.

Additionally required: Diluent 2.

A negative control is included in reagent 2.

Gel agglutination assay for PNH via detection of DAF and MIRL by specific monoclonal antibodies. Cells with these antigens bind the mouse antibodies.

After centrifugation through the gel, cells carrying antibodies, confirming the presence of MIRL or DAF, will show a positive reaction. This denotes that the patient does not have PNH.

Negative results or double population confirm the presence of PNH.

Test characteristics:

• very rapid: result in <30 min
• very simple procedure
• very reliable performance*
• economical: unused microtubes in the card can be used at a later time

* Gupta R, et al. A prospective comparison of four techniques for diagnosis of paroxysmal nocturnal hemoglobinuria. Int J Lab Hematol. 2007;29:119-26

Diag. appl.
A test for the exclusion or confirmation of PNH should be considered in patients with otherwise unexplained thrombembolic complications, or in patients with hemolysis or hemorrhage, anemia, and other symptoms.

Patients with PNH have a defective gene called PIG-A, involved in the biosynthesis of glycosyl-phosphatidylinositol (GPI). Without GPI, important regulatory proteins (e.g. CD55 or “DAF”, and CD59 or “MIRL”) cannot bind to the cell surface and protect blood cells from attacks of complement. This may result in a break down of erythrocytes and release of hemoglobin which causes the urine to turn dark during an episode (or “paroxysm”) of hemolysis, though this is not found in all cases.

PNH is an acquired condition induced by exposure to chemicals or certain antibiotics. In many patients their PNH cells may disappear from the blood after some time. Repeating a test for PNH may help to identify patients who are likely to have a spontaneous cure.

Treatment of PNH includes immune-suppressive drugs, supplementation with iron and folic acid. Blood transfusions or anticoagulation may be needed. Soliris™ (Eculizumab, Alexion Pharmaceuticals, Inc., a humanized antibody against complement C5) is a new drug used to treat PNH*. Eculizumab treatment reduces the risk of clinical thromboembolism in patients with PNH**.

*Rother RP, et al. Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria. Nat Biotechnol. 2007;25:1256-64.

**Hillmen P, et al.. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria.Blood. 2007 1;110:4123-8.

Pkg. size
Profiles or single test
1 x 12
24 Profiles
3 x 1 x 1.4 mL
20 Tests