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Evaluation of Bleeding in Haemophilia Carriers by ISTH-BAT: A Single Center Experience

M. Mortarino1, S.M. Siboni1, I. Garagiola1, M. Boscarino1, F. Peyvandi2

1Angelo Bianchi Bonomi Haemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Milan, Italy, 2Angelo Bianchi Bonomi Haemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Fondazione Luigi Villa, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy

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.

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