Paroxysmal nocturnal hemoglobinuria (PNH), previously Marchiafava–Micheli syndrome, is an hematopoietic stem cell disorder leading to a partial or absolute deficiency of all glycophosphatidyl-inositol (GPI)-linked protein, that causes an increase in sensitivity to complement-mediated cell destruction.
PNH is characterized by chronic intravascular hemolytic anemia, hemoglobinuria, cytopenias due to bone marrow failure, thrombosis and rarely leukemic transformation.
FLAER is used in a flow cytometry test to diagnose paroxysmal nocturnal hemoglobinuria (PNH).
FLAER is produced by labeling an inactive variant of the protein proaerolysin with Alexafluor® 488 succinimidyl ester. The reagent binds selectively to cell surface GPI-anchored proteins, , but will not bind to PNH cells without having that anchor. It is more sensitive than CD59 and detects abnormal small granulocyte populations.
It’s available in liquid and lyophilized format.
Note: FLAER is not suitable for the evaluation of erythrocytes and platelets
