Abstract Number: PB1209
Meeting: ISTH 2020 Congress
Background: Patients with UBD experience spectrum of bleeding symptoms and there is no standardised management.
Aims: Review bleeding patterns and outcomes in patients with UBD.
Methods: Retrospective cohort analysis of patients with UBD at Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London and Coagulation Centre Rhine-Ruhr, Duisburg, Germany.
Results: Out of 4969 patşents referred to both clinics over 10-year period, 162 patients with UBD were identified (3.2% incidence).
All patients underwent full haemostatic assessment with normal results.
109 (68%) were female and 52 (32%) male. Mean age at diagnosis was 25 years (range 2months-84 years). There was only one case of consanguinity.
Leading presenting symptoms in women were heavy-menstrual bleeding (HMB) (32%), haematoma/easy bruising (26%), post-operative bleeding (16%) and epistaxis (15%). Most frequent symptoms leading to diagnosis in men were epistaxis (71%), haematoma/easy bruising, post-operative bleeding and bleeding after tooth-extraction were all equally prevalent (6%).
19% incidence of anaemia (range 8.3-11.9g/dL), all received oral iron.
Mean BAT score was 3.56 (range 1-17) and in women mean PBAC score was 266 (range 68-1085).
24 patients experienced acute bleeding episode requiring A&E attendance, with female:male ratio of 9:1. Postpartum haemorrhage (63%) and HMB (25%) were the commonest emergencies. Blood transfusion was required in 10 patients.
33 patients underwent 59 major/minor surgeries. Haemostatic cover was administered in 38 cases (65%); Tranexamix acid (TXA) (n=27), DDAVP (n=8), platelet transfusion (n=3). There were five cases of post-operative bleeding requiring blood transfusion.
Of women with HMB, 39% suffered from menarche. 30% received TXA, 25% combined hormonal-contraception and 14% combination therapy. Six women required hysterectomy.
Conclusions: Patients with UBD suffer from a variety of bleeding symptoms and are at risk of acute bleeding. Women are more likely to suffer with bleeding symptoms leading to diagnosis, with HMB being their commonest symptom.
To cite this abstract in AMA style:Turan O, Epstein A, Halimeh S, Siebert M, Abdul-Kadir R. Unclassified Bleeding Disorder (UBD)-Bleeding Phenotype and Management [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/unclassified-bleeding-disorder-ubd-bleeding-phenotype-and-management/. Accessed September 27, 2023.
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