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Introduction/Aim. Hemophilia A is an antihemophilic factor deficiency which requires life-long treatment. The aim of this analysis was to present the effects of prophylactic non-factor replacement therapy in ten patients with hemophilia A. Patients and This retrospective analysis was conducted on ten male patients (4 children, 1 adolescent, and 5 adults) with severe hemophilia A and a history of antihemophilic factor replacement prophylaxis, prior to the initiation of emicizumab prophylaxis. A single adult patient developed inhibitors during the course of factor replacement prophylaxis. Four adult patients had already developed hemophilic arthropathy before the initiation of non-factor replacement prophylaxis. Two adult patients received emicizumab prophylaxis every four weeks, while others received emicizumab every two weeks. After a 14-month period (average) of non-factor replacement prophylaxis, we analyzed the number of breakthrough bleeding episodes, annualized bleeding rate, involvement of target joints, adverse drug reactions, and interviewed the patients regarding their satisfaction with the non-factor replacement treatment. Results. None of the patients on emicizumab prophylaxis experienced breakthrough bleeding or clinical worsening of the affected target joints during the period of emicizumab prophylaxis. Annualized bleeding rate was zero in all patients on emicizumab prophylaxis. No adverse drug reactions occurred in our patients during emicizumab prophylaxis. All patients reported greater treatment satisfaction compared to the replacement prophylaxis. Conclusion. By providing safety from bleeding events and potentially the stability of the affected joints, emicizumab prophylaxis enables greater activity and increases the quality of life of treated patients. [ABSTRACT FROM AUTHOR]

Bleeding Assessment Tools (BATs) are bleeding scoring systems used for screening and quantitative assessment of mild bleeding disorders. They consist of a standardized questionnaire and a scoring system that is used for the summation of the final score. In this review article, we have presented BATs that are applied in the area of hematology. The earlier BATs were designed to distinguish patients with von Willebrand disease (VWD) from healthy individuals. Later modifications of the original Vicenza-BAT were developed in order to improve its specificity, precision, and flexibility, as well as to shorten the administration time. The most significant of these modifications is the International Society on Thrombosis and Hemostasis Bleeding Assessment Tool (ISTH-BAT), which is also validated for use in patients affected by hemophilia and inherited platelet disorders. ISTH-BAT score of = 6 in adult females, = 4 in adult males, and = 3 in children is considered abnormal. The WHO developed the first BAT for immune thrombocytopenia (ITP). However, more recently, the ITP International Working Group (IWG) designed the ITP-BAT. The IWG defines a severe or clinically relevant bleeding manifestation as an ITP-BAT SMOG index of S > 2 and/or M > 1 and/or O > 1. In 2016, a group of Chinese experts created a simple modification of the ITP-BAT, the ITP-2016. The values of the ITP-2016 Bleeding Score = 5 indicate severe immune thrombocytopenia. In the primary healthcare setting BATs serve as a valuable screening tool for discovering patients with bleeding disorders, who require further hematologic investigation. [ABSTRACT FROM AUTHOR]

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