Abstract Number: PB0911
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Haemophilia A and B are haemorrhagic disorders characterised by coagulant factors VIII/IX deficiency or dysfunction. Due to X-linked recessive inheritance, males are affected, while female are carriers. Haemophilia carriers may have bleeding symptoms and wide range of clotting factor levels: approximately 30% of carriers express low clotting factor level.
Aims: To evaluate bleeding symptoms by quantitative bleeding assessment tools from ISTH (BAT score) in haemophilia A and B carriers and to correlate BAT score with coagulant factor level.
Methods: Haemophilia carriers, referring to Haemophilia and Thrombosis Center of Milan, with low factor level (≤50 IU/dL) were interviewed by a single physician. BAT score ≥6 has been considered abnormal in adult females.
Results: BAT score was assigned to 65 female carriers. Bleeding symptoms are summerized in the table. Haemarthrosis and muscle haematomas, the most frequent symptoms in haemophilic patients, were reported in 11% and 17% of women, respectively. Carriers with these type of bleeds had a lower coagulant activity than other carriers without these symptoms. The median factor level in carriers with haemarthrosis was 4 IU/dL (IQR: 3-7) versus 37 IU/dL (IQR: 28-44) in carriers without haemarthrosis (p< .001); while in carriers with muscle hematomas, the median factor level was 14 IU/dL (IQR:7-28) versus 38 IU/dL (IQR: 29-44) in carriers without haematomas (p< .001).
Carriers with high BAT score ≥6 showed a median factor level of 28 IU/dL (IQR: 12-37), while haemophilia carriers with BAT score < 6 had a median factor level of 39 IU/dL (IQR: 30-44) (p=.003).
Conclusions: In our study, a significant difference in terms of factor level between the group of carriers with high and low BAT score was found. These findings support the evidence that factor coagulant level, even at the lower end of the normal range in haemophilia carriers, may lead to increased bleeding manifestations.
| menorrhagia | 46/62 (74%) |
| cutaneous | 45/65 (69%) |
| tooth extraction | 26/46 (54%) |
| epistaxis | 28/65 (43%) |
| bleeding post surgery | 20/48 (42%) |
| post-partum hemorrhage | 10/41 (24%) |
| muscle hematomas | 11/64 (17%) |
| hemarthrosis | 7/65 (11%) |
| gastrointestinal bleeding | 3/64 (5%) |
| hematuria | 5/65 (3%) |
[Table. Bleeding symptoms of haemophilia A and B carriers]
To cite this abstract in AMA style:
Mortarino M, Siboni SM, Garagiola I, Boscarino M, Peyvandi F. Evaluation of Bleeding in Haemophilia Carriers by ISTH-BAT: A Single Center Experience [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/evaluation-of-bleeding-in-haemophilia-carriers-by-isth-bat-a-single-center-experience/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/evaluation-of-bleeding-in-haemophilia-carriers-by-isth-bat-a-single-center-experience/