Haemophilia, severity and bleeds
Being aware of the impact haemophilia can have on everyday life, understanding prevention and engaging with your healthcare team can help you better work towards personal goals and achievements.
Why do bleeds occur?
Haemophilia affects the blood’s ability to clot.1,2 It is caused by a lack or low levels of clotting factors in the blood – either factor VIII in haemophilia A or factor IX in haemophilia B.1–3 These clotting factors are essential for the formation of blood clots, which stop bleeding.1 When these factors are deficient or absent, the body cannot clot blood effectively, leading to uncontrolled bleeding episodes.4
The severity of haemophilia is determined by clotting factor levels in the blood and directly impacts the frequency and severity of bleeding episodes.2,6,7
What do factor levels indicate?
Factor levels can help show the severity of haemophilia, depending on how much of the clotting factor a person has in their blood compared to the baseline level.1,2,5–7
Factor levels <1% (Severe haemophilia)
Significant risk of bleeding, which occurs frequently, even with minor injuries or sometimes with not injury at all (spontaneous bleeding). Joint and muscle bleeds are common, and internal bleeding can happen in various tissues of the body.
Factor levels between 1–5% (Moderate haemophilia)
Occasional spontaneous bleeding is possible. Excessive bleeding may occur after trauma. Bleeding may be triggered by injuries that would normally cause only minor bleeding in people without haemophilia.
Factor levels between 5–<40% (Mild haemophilia)
Spontaneous bleeding is rare. Bleeding may occur after significant trauma or surgery.
Factor levels >40% (Normalised haemostasis)
With appropriate care, factor levels in the blood may increase and may become similar or the same as a person without haemophilia.
Types of bleeds in haemophilia
The type of bleed a person with haemophilia experiences can vary based on the severity of the condition.6,7 Bleeding can happen in different ways and locations, and the nature of the bleeding depends on the amount of clotting factor available in the blood.8
Haemophilia patients may experience different types of bleeding:9–12
- External bleeding: This occurs on the skin's surface or in the mouth, such as from cuts, scrapes or biting the mouth, lips or tongue
- Internal bleeding, including within soft tissue: Bleeding that happens inside the body, such as in muscles or joints
- Post-surgical bleeding: After an operation, there is an increased risk of excessive bleeding occurring
- Spontaneous bleeding: Bleeding without a cause, often happening in the joints or muscles
- Silent bleeds: Small, unnoticed internal bleeds that may not show any symptoms but can still cause damage mainly into joints
What are silent bleeds?
Silent bleeds are small, unnoticed internal bleeds that occur in the tissue and don’t cause visible symptoms but can have serious long-term effects. These tiny internal bleeds occur in areas like tissues, muscles, or joints.13,14
Silent bleeds can cause cumulative damage over time.13,14 While they might not cause pain or be visible at first, untreated silent bleeds can lead to joint conditions like synovitis (inflammation of the joint lining), which results in pain, swelling, and impaired mobility.13,14
It's important to understand how to detect silent bleeds, as they can cause lasting harm and make it harder to move and perform daily activities.8,13
Spotting silent bleeds can be difficult, as they do not occur with symptoms. Your doctor should periodically assess your joint status to identify any abnormalities that can be caused without an observed bleed and may carry out a physical examination to help identify signs of bleeding using imaging tools such as ultrasound or magnetic resonance imaging (MRI) to detect joint abnormalities that are not visible externally.13,15,16
Common bleeding sites in haemophilia
Bleeding can occur in various parts of the body. The sites where bleeding may occur vary based on severity. However, some common sites include:2,8
- Joints:
- Each bleed is presented by pain and swelling. Frequent bleeds lead to joint damage and chronic pain
- Muscles:
- Internal bleeding in muscles are presented by swelling, pain, and bruising. Muscle bleeds can also affect mobility and cause discomfort
- Skin:
- Bleeding from small cuts may take longer to stop; even minor injuries can cause excessive bleeding
- Nose:
- Nosebleeds are common, can happen frequently, and may take longer to stop than in people without haemophilia
Managing a haemophilia bleed
When a bleed happens, it’s important to treat it right away to minimise damage.8,17 Early treatment helps:8,17
- Stop the bleeding and prevent it from getting worse.
- Limit damage to tissues, muscles, and joints
- Reduce the impact of the bleed on daily life, allowing for quicker recovery
Bleeding can be managed by using First Aid steps known as RICE — this stands for Rest, Ice, Compression and Elevation.2,8 Ask your healthcare provider about these steps.
Treatment depends on the location and severity of the bleed. For severe bleeds, immediate medical attention is necessary.8
Treatment will likely involve clotting factor replacement therapy, where the missing clotting factor is given through an injection to help the blood clot properly.8
In addition to clotting factor therapy, other treatments may be used to help manage bleeding and its consequences, such as:8
- Pain relievers (analgesics): To reduce pain from the bleed, especially in joints and muscles
- Supportive care: Including physical therapy or other interventions to aid in recovery
- World Federation of Hemophilia. What is Hemophilia? Available at: https://www1.wfh.org/publications/files/pdf-1324.pdf. Accessed February 2025.
- The Haemophilia Society. Understanding haemophilia. Available at https://haemophilia.org.uk/wp-content/uploads/2023/07/THS_A5_understanding_JCv1_interactive.pdf. Acessed February 2025.
- Castaman G and Matino D. Haematologica 2019;104(9):2–9.
- The Haemophilia Society. Rare bleeding disorders. Available at: https://haemophilia.org.uk/wp-content/uploads/2023/07/THS_A5_RareDiseases_JC_website.pdf. Accessed February 2025.
- Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A, et al. J Thromb Haemost 2014;12:1935–1939.
- National Bleeding Disorders Foundation. Hemophilia A. Available at: https://www.bleeding.org/bleeding-disorders-a-z/types/hemophilia-a. Accessed February 2025.
- National Bleeding Disorders Foundation. Hemophilia B. Available at: https://www.bleeding.org/bleeding-disorders-a-z/types/hemophilia-b. Accessed February 2025.
- Srivastava A, Santagostino E, Dougall A, Kitchen S, Sutherland M, Pipe SW et al. Haemophilia 2020;26(Suppl 6):1–158.
- National Bleeding Disorders Foundation. Types of bleeds. Available at: https://www.bleeding.org/bleeding-disorders-a-z/overview/types-of-bleeds. Accessed February 2025.
- Kleiboer B, Layer MA, Cafuir LA, Cuker A, Escobar M, Eyster ME, et al. J Thromb Haemost 2022;20(4):857–865.
- Mayo Clinic. Hemophilia. Available at: https://www.mayoclinic.org/diseases-conditions/hemophilia/symptoms-causes/syc-20373327#. Accessed February 2025.
- Gooding R, Thachil J, Alamelu J, Motwani J, Chowdary P. J Blood Med 2021:12:209–220.
- Skinner MW, Nugent D, Wilton P, O’Mahony B, Dolan G, O’Hara J, et al. Haemophilia 2020;26(1):17–24.
- Van Meegeren MER, Roosendaal G, jansen NWD, Lafeber FPJG, Mastbergen SC. Cartilage 2013;4(4):313–320.
- Wilkins RA, Stephensen D, Siddle H, et al. BMJ Open. 2022;12(1):e052358.
- Zwagemaker AF, Kloosterman FR, Hemke R, et al. J Thromb Haemost 2022;20(5):1126-1137.
- Mehta P, Reddivari AKR. Hemophilia. [Updated 2023 Jun 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK551607/.
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