Hemophilia treatment relies upon replacement of the deficient factor to restore physiological
levels in plasma. The development of inhibitors is the main complication of replacement
therapy, which renders replacement therapy ineffective and requires the use of alternative
hemostatic drugs known as bypassing agents. The hemostatic response to bypassing agents
is different from patient to patient and even in the same patient during different
bleeding episodes. Up to now, no routine laboratory test has been found suitable to
monitor efficacy and safety of these drugs. The unpredictable clinical response to
bypassing therapy and the lack of a monitoring laboratory tool renders surgery in
inhibitor patients a big challenge for the risk of both bleeding and thromboembolic
complications. The thrombin generation assay (TGA) has been proposed as a monitoring
tool in this patient population on the basis of the results obtained both in vitro
and ex vivo. This review aims to summarize the current published evidence on the use
of TGA as a laboratory monitoring tool in patients with hemophilia complicated by
inhibitors.
Keywords
hemophilia - inhibitors - thrombin generation - bypassing therapy
