title: Diagnostic significance of measurement of the receptor for urokinase-type plasminogen activator on granulocytes and in plasma from patients with paroxysmal nocturnal hemoglobinuria.
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired stem cell disorder characterized by the deficiency of all proteins anchored to the membrane by the glycosyl-phosphatidylinositol (GPI) anchor. The receptor for urokinase-type plasminogen activator (uPAR) also is attached to the cell membrane by a GPI anchor, and that soluble uPAR (suPAR) is present in plasma. In the present study, we measured uPAR, CD55, and CD59 on granulocytes by means of flow cytometry and suPAR in plasma by means of immunoradiometric assay. The subjects were 20 patients with PNH, 59 other patients with anemia, and 21 healthy individuals. In patients with PNH, both the mean fluorescence intensity and the positive percentage of fluorescence-activated granulocytes of uPAR, CD55, and CD59 were remarkably decreased, whereas in patients with other forms of anemia, except 2 patients with aplastic anemia, the results were not altered in comparison with those for the healthy individuals. The level of uPAR was reduced to the same extent as were those of CD55 and CD59 on the PNH-affected granulocytes. Some peak shape abnormalities (double peaks, peak tailing, or both) in the histogram of fluorescence intensity were also found in patients with PNH. The suPAR concentration of PNH plasma was 4.04+/-2.47 ng/mL, which was higher than that of the healthy individuals, 1.73+/-0.96 ng/mL (P < .01). The positive percentage of fluorescence-activated granulocytes was inversely associated with the plasma suPAR level in patients with PNH (r = -0.79, P < .01). Our data suggest that measurement of uPAR on granulocytes by means of flow cytometry and of suPAR in plasma by means of immunoradiometric assay are specific techniques for the diagnosis of PNH.
authors.affiliation: Thrombosis and Hemostasis Research Unit, Jiangsu Institute of Hematology, First Affiliated Hospital of Suzhou University, People's Republic of China.
authors.forename: Weiqiang
authors.initials: W
authors.lastname: Gao
authors.forename: Zhaoyue
authors.initials: Z
authors.lastname: Wang
authors.forename: Xia
authors.initials: X
authors.lastname: Bai
authors.forename: Yuyun
authors.initials: Y
authors.lastname: Li
authors.forename: Changgeng
authors.initials: C
authors.lastname: Ruan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal; D018958:CD55 Antigens / Q000032:analysis; D018957:CD59 Antigens / Q000032:analysis; D016022:Case-Control Studies; D005260:Female; D006098:Granulocytes / Q000378:metabolism*; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007118:Immunoassay; D008297:Male; D008875:Middle Aged; D011956:Receptors, Cell Surface / Q000032:analysis* / Q000097:blood / Q000276:immunology; D055293:Receptors, Urokinase Plasminogen Activator; D012995:Solubility
chemical_list: D000911:Antibodies, Monoclonal; D018958:CD55 Antigens; D018957:CD59 Antigens; C524898:PLAUR protein, human; D011956:Receptors, Cell Surface; D055293:Receptors, Urokinase Plasminogen Activator