Does the intensity and quality of treatment and not only the factor VIII level influence global hemostasis in patients with hemophilia A?

Berndtsson, Maria; Zetterberg, Eva; Holmström, Margareta; Soutari, Nida Mahmoud Hourani; Miković, Danijela; Elezović, Ivo; Antović, Jovan P.

doi:10.1016/j.thromres.2016.05.024

Cited by 1 publication

(1 citation statement)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The overall fibrinolytic potential (OFP, %) is the difference between OCP and OHP values, and is expressed as a percentage of the OCP. Figure 1A illustrates all parameters measured by this method but, as OHP was previously the most examined parameter in HA patients, 10,18 only OHP results were presented and analyzed in this study.…”

Section: Methodsmentioning

confidence: 99%

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

Miloš

Herak

Soutari

et al. 2020

Int J Lab Hematology

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Section: Methodsmentioning

confidence: 99%