Thromboelastography in children with coagulation factor deficiencies

Abstract

Summary Hemophilia is traditionally classified according to the levels of the deficient coagulation factor as Severe (<1%), Moderate (1–5%) or Mild (>5%). However, it is well known that the factor activity does not necessarily correspond to the clinical bleeding manifestations. As prophylactic therapy is the best method of prevention of serious complications such as hemophilic arthropathy, a test that may predict the bleeding pattern would be extremely beneficial. Thromboelastography (TEG) uses whole blood to determine clot formation characteristics, such as initiation, propagation as well as strength of the clot, and is now being extensively studied in bleeding and thrombophilia. This study attempted to determine the TEG characteristics in 47 children with moderate hemophilia (MH) and severe hemophilia with (SHI) and without inhibitors (SH) and tried to retrospectively correlate them to the clinical bleeding patterns. TEG showed evidence of faster and better clot formation, as evidenced by a higher maximum thrombin/fibrin generation, in those with mild bleeding manifestations compared to those with severe bleeding tendency, in addition to the expected prolongation in time to formation of clot related to factor deficiency. This may be a potentially useful tool to evaluate the bleeding tendency and determine need for prophylaxis in children with hemophilia.

Comparing bleed frequency and factor concentrate use between haemophilia A and B patients

“…[4][5][6]. Thrombin generation may show a greater correlation with the phenotypic expression of bleeding in these patients [4,5].…”

A Standardized Technique for Performing Thromboelastography in Rodents

“…The importance of developing a standard method of performing TEG is to improve reproducibility and facilitate comparison of results in the literature and between investigators. TEG is a versatile and comprehensive tool which measures specific components of coagulation, increasing its use in diverse clinical settings such as cardiac surgery (3), liver transplant (2), trauma (4), sepsis (14), and hemophilia (15). Because TEG can function in multiple clinical arenas, and relies on proper performance by the operator, following a standard technique and choosing the appropriate type of TEG to perform are critical in achieving consistent results.…”

Aptamer ARC19499 mediates a procoagulant hemostatic effect by inhibiting tissue factor pathway inhibitor

“…9,30 Treatment of normal whole blood with the anti-FVIII antibody induced a hemophilia A-like state, characterized by a significantly prolonged R value and a decreased angle ( Figure 5B). The addition of ARC19499 to the antibody-treated blood resulted in a dose-dependent decrease in the R value, reaching levels observed with untreated blood with 100nM aptamer ( Figure 5B).…”