Naslov
Overall hemostasis potential and aPTT-clot waveform analysis as powerful laboratory diagnostic tools for identification of hemophilia A patients with unexpected bleeding phenotype

Autori
Miloš Marija ; Coen Herak Désirée ; Mahmoud Hourani Soutari Nida ; Pavić Josipa ; Zupančić-Šalek Silva ; Zadro Renata ; Antović Jovan P.

Izvornik
International journal of laboratory hematology (1751-5521) 43 (2021); 273-280

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
aPTT-clot waveform analysis, bleeding phenotype, endogenous thrombin potential, hemophilia A, overall hemostasis potential

Sažetak
Introduction Traditionally used laboratory methods do not always accurately reflect bleeding severity in hemophilia A (HA) patients. The ability of three global assays for identifying patients with unexpected bleeding phenotype was investigated. Methods Overall hemostasis potential (OHP), aPTT-clot waveform analysis (aPTT-CWA), endogenous thrombin potential (ETP), FVIII activities, and prothrombin fragment 1 + 2 concentrations were measured in 62 HA patients (30 severe and 32 non-severe) and 27 male controls. Bleeding phenotype was determined using our proposed scoring system including age at first joint bleed, number of target joints, and number of joint/muscle bleeds per year. Bleeding score ≤ 4 defined patients with mild bleeding phenotype (N = 27) ; score ≥ 5 defined severe bleeding phenotype (N = 35). Results The receiver operating characteristic analysis performed for distinguishing patients with severe and mild bleeding phenotype yielded following values of area under the curve: 0.910 (FVIII) ; 0.891 (aPTT-CWA parameter DELTA) ; 0.769 (OHP) ; and 0.634 (ETP). Unexpected bleeding phenotype was identified in 11/62 HA patients: 8/32 (25%) non– severe HA patients had severe, while 3/30 (10%) severe HA patients had mild bleeding phenotype, and global assays enabled the identification of all these patients. OHP and DELTA were revealed as the most reliable parameters for bleeding phenotype determination (10/11 and 9/11 unexpected results, respectively). Conclusion This study emphasizes OHP and aPTT-CWA as a powerful laboratory diagnostic tool in identifying HA patients with unexpected bleeding presentations, with the best results achieved by combining both assays. Global assays should not completely replace FVIII activity measurement but should be a part of the HA diagnostic algorithm.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti, Biotehnologija u biomedicini (prirodno područje, biomedicina i zdravstvo, biotehničko područje)

POVEZANOST RADA