Abstract Number: PB0909
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Patients with mild haemophilia tend not to develop spontaneous haemarthrosis and usually only need factor replacement at times of trauma or surgery. Haemarthrosis commonly occurs in knee and ankle joints; hip bleeds are rare.
We have a cohort of patients with mild haemophilia A who have sustained hip bleeds.
Aims: To describe and quantify hip bleeds in children with mild haemophilia A in a single paediatric comprehnsive care centre.
Methods: A retrospective case note review was carried out of all boys with mild haemophilia A who had reported hip bleeds in the preceding 8 years at a single paediatric comprehensive care centre. The hip bleeds were either diagnosed clinically, by ultrasound or aspiration. Demographics, mechanism of injury, diagnostics, treatment and joint status were explored.
Results: Of a current cohort of 58 patients with mild haemophilia A, seven hip bleeds were identified, including two for whom this was their diagnostic event. Mean age was 7.1 years (3.7years-11.2years) and mean baseline factor VIII level was 15IU/dl (8IU/dl-25IU/dL). See table1 for full baseline demographics and diagnostics.
Treatment was variable: two patients received no specific haemostatic therapy; two were initially treated with DDAVP followed by recombinant factor VIII; three received recombinant factor VIII only. Treated patients received a mean 4.5 days (range 2-7 days) of factor replacement.
Follow up from bleed to most recent physio review was an average of 27 months (range 1- 79). To date no pain, persistent restriction or joint impairment was identified in any patient.
Conclusions: In this review boys with mild haemophilia A between 3 to 11years appear at risk of developing spontaneous hip bleeds. To date there appears to be no impact on joint health or function in our cohort and suveillance continues. This has led to a change in our counselling practice around hip bleed recognition for families with mild haemophilia.
Characteristic | N = 7 |
Baseline factor (IU/dL) mean (range) |
15 (8 – 25) |
Age at which hip bleed occurred (range) |
7 years 1 month (3 years 8 months – 11 years 2 months) |
Weight (Centile) mean (range) |
56th (3rd-86th) |
Mechanism of injury Spontaneous – no obvious trauma Minimal trauma – recent sport activity but no obvious trauma Trauma – clear mechanism of injury |
4 2 1 |
Diagnosis of bleed Clinical suspicion – Experience paediatric haemophilia physiotherapist Ultrasound – Joint effusion Aspiration – Haemarthrosis |
1 4 |
Delay in presentation (days) mean (range) *excluding 2 patients for who this was their diagnostic event |
1.8 (0 – 5)* |
[Table 1. Summary of patient demographics and diagnostics]
To cite this abstract in AMA style:
Gosrani D, Bladen M, Hubert N, Merchant R, Masterton G, Badle S, Taylor A, Sibson K, Spires J, Efford J, Barrie A, Mathias M, Liesner R. Hip Bleeds in Boys with Mild Haemophilia A: A Single Centre Experience [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/hip-bleeds-in-boys-with-mild-haemophilia-a-a-single-centre-experience/. Accessed March 21, 2024.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/hip-bleeds-in-boys-with-mild-haemophilia-a-a-single-centre-experience/