Abstract Number: PB0948
Meeting: ISTH 2021 Congress
Background: Von Willebrand disease (VWD) is the most common congenital bleeding disorder. Its main symptom is recurrent bleeding from mucosal tissues, which can include heavy menstrual bleeding (HMB), even leading to anaemia and the need for hospitalisation and transfusion with red cell concentrates (RCC).
Aims: To describe case studies illustrating appropriate patient management in HMB.
Methods: Three cases of teenagers with VWD and HMB are presented.
Results: The first patient is a 14.5-year-old girl, who started experiencing heavy menstrual bleeding when she was 13. Coagulation parameters (APTT, INR, fibrinogen) were always normal. Due to substantial anaemia, the patient often received RCC. Contraceptive treatment was introduced, but was unsuccessful and VWD started being suspected. Laboratory tests revealed FVIII levels of 51%; VWF:RCo levels of 14.5% and VWF:Ag of 18.3%. VWD type 2 was diagnosed, which led to end the contraception treatment and start intermittent prophylaxis with pdVWF/FVIII (ratio 2.4:1) for 3 days (40 IU VWF/kg/day). This normalized the menstruation.
The second patient is a 14-year-old girl, whose HMB even required hospitalisation. Contraceptives had no effect, which elicited a suspicion of VWD. Laboratory tests showed FVIII levels of 61.5%, VWF:RCo of 20.8% and VWF:Ag of 36.3%. After obtaining the results, VWD type 2 was confirmed and pdVWF/FVIII was introduced prophylactically during the first 2 days of the menstrual period (40 IU VWF/kg/day). This normalized the menstruation.
The third patient is a 16-year-old girl with VWD type 3. From her first menstrual period, prolonged bleeding was observed, together with the development of substantial anaemia. After introducing pdVWF/FVIII (40 IU VWF/kg/day) the first three days of each period, menstrual bleeding was normalized.
Conclusions: In the case of recurrent HMB despite normal coagulation parameters in the blood test results, one should always suspect VWD. Intermittent prophylaxis with VWF-containing concentrates can be considered in these cases.
To cite this abstract in AMA style:Laguna P, Laguna A, Matysiak M. Intermittent Prophylaxis for Heavy Menstrual Bleeding in Von Willebrand Disease [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/intermittent-prophylaxis-for-heavy-menstrual-bleeding-in-von-willebrand-disease/. Accessed September 16, 2021.
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