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Updated: May 15, 2026
08:13
Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice
Published on: September 30, 2021
Outcome in moderate haemophilia
Ingrid den Uijl1, Douwe Biesma2, Diederick Grobbee3
1van Creveldkliniek, Department of Haematology, Medical University Centre Utrecht, Utrecht, The Netherlands Julius Center for Health Sciences and Primary Care, Medical University Centre Utrecht, Utrecht, The Netherlands.
Blood Transfusion = Trasfusione Del Sangue
|December 19, 2012
Summary
Moderate haemophilia patients typically experience few bleeds, but some require prophylaxis. Early joint bleeds before age five may predict the need for prophylactic treatment in moderate haemophilia.
Area of Science:
- Hematology
- Internal Medicine
- Pediatrics
Background:
- Moderate haemophilia is a rare bleeding disorder.
- Assessing outcomes and treatment associations in this population is crucial.
Purpose of the Study:
- To evaluate short- and long-term outcomes in patients with moderate haemophilia.
- To investigate the relationship between treatment and outcomes in this rare condition.
Main Methods:
- Study included 75 moderate haemophilia patients (1-5% factor activity) without inhibitors.
- Collected lifelong bleeding and treatment data; assessed joint health using the Haemophilia Joint Health Score (HJHS).
- Adults completed questionnaires on activity and quality of life (HAL, SF-36, EQ5D).
Main Results:
- Median age was 37 years; 89% had haemophilia A.
- Low bleeding frequency (median 2.0 bleeds/year), with good joint function (82% scored <10 on HJHS).
- 29% received prophylaxis, often linked to early joint bleeds (before age 5).
Conclusions:
- Most moderate haemophilia patients have minimal complications.
- A subset requires prophylactic treatment, identifiable by early-onset joint bleeding.
- Early intervention may be key for patients with a more severe bleeding pattern.
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