Keywords
Hemolytic test
eculizumab
Complement blockade
How to Cite
Abstract
The diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH) can be made with different methodologies, the most used are flow cytometry (Gold standard) and the HAM test.
The rationale for this latter test is based on the susceptibility of PNH erythrocytes to lysis against both normal and patient acidified human serum. This test is considered positive and diagnostic when there is lysis in both tubes.
The great advance in the treatment of this disease is due to Eculizumab, a monoclonal antibody directed against the C5 fraction of the complement, which prevents the formation of the MAC (Membrane Attack Complex).
To monitor the response to treatment with Eculizumab, a hemolytic test was designed, which we call the MET test (Monitoring Eculizumab Test).
It is a simple, accessible and economical methodology that can be used to:
(a) Monitor the efficacy of treatment with Eculizumab.
(b) Monitor the response to the increased dose of Eculizumab.
(c) Detect patients who are refractory to treatment.
Monitoring with MET thus makes it possible to adjust the doses and/or the intervals of drug administration for a better therapeutic response.
References
Parker C, Omine M, Richards S et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709.
Brodsky RA. Paroxysmal nocturnal hemoglobinuria. Blood. 2014;124(18):2804-2811.
Loschi M, Porcher R, Barraco F et al. Impact of eculizumab treatment on paroxismal nocturnal hemoglobinuria: a treatment versus no treatment study. Am J Hematol. 2016;91(4):366-370.
Arcavi M, Ceballo F, Caracciolo M, Lazarowski A. Paroxysmal nocturnal hemoglobinuria: Test to monitor the action of eculizumab treatment. Lab Hematol.
;00:1–6.
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