Skip over main navigation
  • Log in
  • Basket: (0 items)
  • Perth Blood Institute
  • Thrombosis Australia
  • Thrombosis Australia Professionals
The Perth Blood Institute Limited
  • Search
  • Facebook
  • Linkedin
  • Email
  • Search
  • Facebook
  • Linkedin
  • Email
Login Register Subscribe Donate
Menu
  • Who we are
    • Vision, mission and values
    • What we do
    • Our facilities
    • Our team
      • Board
      • Investigators
      • Thrombosis Australia Advisory Panel
    • Work with us
  • Our clinical trials
    • Clinical trials
    • Collaborations
    • Participate in a clinical trial
    • Useful links
  • Our research
    • Current research projects
    • Research highlights
    • Research collaborations
      • PBI
      • APTIN
    • Participate in a research project
    • Published research
  • About blood disorders
    • Overview
    • Blood disorders
      • Anaemia
      • Antiphospholipid syndrome (APS)
      • Haemophilia
      • Polycythemia vera
      • Thrombocythaemia
      • Thrombophilia
      • TTP
      • von Willebrand Disease
    • Blood cancers
      • Myelofibrosis (MF)
      • Chronic lymphocytic leukaemia
      • Lymphoma
    • For patients and families
  • Personal stories
    • Living with a blood disorder
  • Get involved
    • Donate
    • Fundraise
    • Partner with us
    • Leave a gift in your will
    • Other ways to help
  • News and events
    • Latest news & info
    • Previous news
    • 10 Year Anniversary
    • Significant days
      • 2023
      • 2022
    • Media
    • Upcoming events
    • Publications
  • COVID-19
  • Admin
    • Log in
    • Perth Blood Institute
    • Thrombosis Australia
    • Thrombosis Australia Professionals
  • Basket: (0 items)
  • Haemophilia
  1. About blood disorders
  2. Blood disorders

Haemophilia

Haemophilia is a rare blood disorder where the blood cannot clot normally because it doesn't have enough blood-clotting proteins/factors. This means a person with haemophilia can potentially bleed for a longer time after a major cut or injury than a person whose blood is able to clot properly.

A severe form of the condition can cause bleeding inside your body, mainly in your knees, ankles and elbows. Internal bleeding can damage your organs and tissues and be life-threatening.

Types of Haemophilia

Haemophilia A – lacks the clotting factor VIII

Haemophilia B – has a deficiency of factor IX

Both occur primarily in males as it is a sex-linked or X-linked disorder

  • the son of a female carrier has a 50% chance of having the disorder.
  • the daughter having a 50% chance of being a carrier.
  • the daughters of men with haemophilia are carriers, however the sons are normal.

It is possible for female carriers to have mild symptoms and show some signs of bleeding. Acquired haemophilia has been reported due to the development of autoantibodies toward factor IX.

Symptoms

Haemophilia A and B have similar clinical features and symptoms vary depending on level of factor deficiency:

  • Bruising easily.
  • Less than 1% - frequent spontaneous bleeding from early life.
  • 1-5% - severe bleeding following injury and occasional spontaneous episodes.
  • Excessive bleeding into the joints.
  • Spontaneous joint bleeding in up to 80% of patients with haemophilia.
  • Above 5% - mild disease associated with bleeding only after injury or surgery.
  • Recurrent bleeding into 'target joints' can progressively lead to pain and restriction of movement and potentially irreversible structural damage.
  • Mortality associated with haemophilia A is common among individuals with cancer and heart disease.

Treatment

The main treatment for Haemophilia A and B is replacing or increasing the clotting factors VIII and IX. This can be achieved using either donated blood or a modified clotting factor made up of cells from two or more sets of DNA (a chimera) which is produced in a laboratory.

Facts about Haemophilia

The following statistics is the reported findings from the Australian Bleeding Disorders Registry and demonstrates minor increases over the period.

2011–2012

  • 2,316 patients with Haemophilia A; 724 patients with severe Haemophilia A
  • 544 patients with Haemophilia B; 102 patients with severe Haemophilia B

2019–2020

  • 2,449 patients with Haemophilia A; 706 patients with severe Haemophilia A
  • 585 patients with Haemophilia B; 112 patients with severe Haemophilia B

2020-2021

  • 2529 patients with haemophilia A; 725 patients with severe haemophilia A
  • 601 patients with haemophilia B; 111 patients with severe haemophilia B

The Federation of Haemophilia Annual Global Survey 1999-2018 presents large increases of cases universally over nearly 2 decades.

  • Haemophilia A – 53,864 (1999); 173,711 (2018)
  • Haemophilia B – 11,591 (1999); 34,289 (2018)

The latest data shows the prevalence of Haemophilia B among males is approx. 15% of all cases (1 in 40,000); and for Haemophilia A it is about 85% (1 in 5,000).


References

  • https://www.cdc.gov/ncbddd/hemophilia/data.html#:~:text=Hemophilia%20A%20affects%201%20in,with%20hemophilia%20A%20each%20year.
  • https://www.blood.gov.au/system/files/documents/abdr-annual-report-2011-12-20140423.pdf
  • https://www.blood.gov.au/sites/default/files/ABDR-Annual-Report-2019-20-FINAL.pdf
  • https://www.mayoclinic.org/diseases-conditions/hemophilia/diagnosis-treatment/drc-20373333
  • Hemophilia B - StatPearls - NCBI Bookshelf (nih.gov)
  • The World Federation of Hemophilia Annual Global Survey 1999‐2018 - Stonebraker - 2020 - Haemophilia - Wiley Online Library
  • Kumar, P., & Clark, M. (2005). Clinical Medicine Sixth Edition, London, UK: Elsevier Saunders
  • Wilding, J. et al. (2018). Obesity in the global haemophilia population: prevalence, implications and expert opinions for weight management. Obesity Reviews, 19(11)

 

Published: 5th September, 2022

Updated: 31st March, 2023

Author: Gina Rodgers

Share this page
  • Email
  • Facebook
  • Twitter

Latest

  • World Heart Day 2023

    World Heart Day 2023

    September 29th is World Heart Day. It is important to KNOW your heart and TAKE CARE of your heart.

  • In memory of Graham

    In memory of Graham

    Graham Wild, a passionate 15-year-old boy with a deep love for nature and animals, he had dreams of running an animal sanctuary. After returning from a trip to see his dad, Graham started to have trouble breathing and his health rapidly deteriorated. Despite urgent medical attention, his heart stopped, and he couldn't be revived. Graham's mother Kathryn would like to share his story.

  • Blood Cancer Awareness Month 2023

    Blood Cancer Awareness Month 2023

    Blood cancers are a complex group of cancers which can affect anyone, at any age, and at any time throughout their lives. September is Blood Cancer Awareness Month and here we present some information about this type of cancer.

  • Lymphoma

    Lymphoma

    Lymphoma or lymphatic cancer is a disease which attacks white blood cells and affects not only the organs associated with the lymphatic system but can impact other organs in the body. Here we provide some background information associated with this type of blood cancer.

Related

  • Thrombocythaemia

    Thrombocythaemia

    Thrombocythaemia and thrombocytosis are blood disorders involving platelets, which can cause blood clots and bleeding.

  • Polycythemia vera

    Polycythemia vera

    Polycythemia vera is a rare type of blood cancer known as a ‘myeloproliferative disorder’ which refers to when bone marrow produces blood cells in a rapid and uncontrolled way.

  • Antiphospholipid syndrome (APS)

    Antiphospholipid syndrome (APS)

    Antiphospholipid syndrome (APS) is a rare acquired pre-thrombotic autoimmune disease. Here we provide some information regarding the condition.

  • Anaemia Fact Sheet

    Anaemia Fact Sheet

    Anaemia is a serious global health concern, predominantly affecting children and pregnant women. The World Health Organization (WHO) estimates that 42% of children under age 5 and 40% of pregnant women around the world suffer from anaemia. Learn more here.

  • von Willebrand Disease (VWD)

    VWD is an inherited bleeding disorder and is one of the most common bleeding disorders worldwide. Here we provide some background information about the disease.

  • Thrombophilia

    Thrombophilia

    Thrombophilia is an abnormal coagulation process which can be inherited or acquired. It results in a hypercoagulable state and increases the risk of thrombotic events where blood clots can form in either the arteries or veins.

  • TTP (Thrombotic Thrombocytopenic Purpura)

    TTP (Thrombotic Thrombocytopenic Purpura)

    TTP is a rare life-threatening blood disorder with an annual incidence of 3 to 11 cases per million people. Here is a review of the condition.

Most read

  • What is thrombosis?

    What is thrombosis?

    Click here to learn more about thrombosis.

  • What are DVT & PE?

    What are DVT & PE?

    Click here to learn more about DVT and PE.

  • Work and Thrombosis

    Work and Thrombosis

    Click here to learn more about how work can increase your risk of thrombosis, and how to prevent it.

  • Travel and Thrombosis

    Travel and Thrombosis

    Click here to learn about how travel can increase your risk of developing dangerous blood clots.

  • Antiphospholipid Antibody Syndrome

    Antiphospholipid Antibody Syndrome

    Click here for information about antiphospholipid antibody syndrome and how it can lead to thrombosis.

  • Contraception and Thrombosis

    Contraception and Thrombosis

    Click here to learn more about how certain contraceptives can increase your risk of dangerous blood clots.

  • Inherited Thrombophilias

    Inherited Thrombophilias

    Click here for information about genetic conditions that can increase the chances of thrombosis.

  • Investigators

    Investigators

  • Participate in a clinical trial

    Participate in a clinical trial

    PBI's Clinical Trials Unit actively runs numerous clinical trials and performs state-of-the-art research into bleeding and clotting disorders and blood cancers.

  • Pregnancy and Thrombosis

    Pregnancy and Thrombosis

    Click here to learn about how pregnancy can increase your risk of thrombosis.

Tag cloud

Acute Myeloid Leukaemia ADAMTS13 blood clots cancer contraceptive Follicular Lymphoma haemophilia A Microangiopathic Thrombocytopenia multiple myeloma oral contraceptive pregnancy Relapsed Multiple Myeloma small vessels thrombosis Thrombotic Thrombocytopaenic Pupura TMA
Donate today

Donate today

Please help to make blood disorders a thing of the past. Every amount is vital to our research, no matter how large or small. Read more

Donate Fundraise

Published: 18th February, 2020

Updated: 8th May, 2023

Author:

SUPERNOVA clinical trial

SUPERNOVA clinical trial

Perth Blood Institute (PBI) start new Australian-wide clinical trial – SUPERNOVA Read more

Published: 3rd July, 2023

Updated: 2nd August, 2023

Author: Gina Rodgers

Comments: 1

PBI celebrates 10 Year Anniversary!

PBI celebrates 10 Year Anniversary!

"It is with your support that we have continued to grow over the past 10 years – to continue to advance medical discoveries and improve care for patients. " Professor Ross Baker Read more

Published: 4th July, 2023

Updated: 21st September, 2023

Author: Bec Morgan

Graham Wild

Graham Wild

Graham Wild, a passionate 15-year-old boy with a deep love for nature and animals, had dreams of running an animal sanctuary. After returning from a trip to see his dad, Graham started to have trouble breathing and his health rapidly deteriorated. Despite urgent medical attention, his heart stopped, and he couldn't be revived. Graham's mother Kathryn would like to share his story. Read more

Published: 13th July, 2023

Updated: 14th September, 2023

Author: Jacqui Le

Sign up for our newsletter

Please enter your first name
Please enter your last name
Please enter your email address Please enter a valid email address (e.g. [email protected])

Helpful links

  • Sitemap
  • Accessibility
  • Terms & conditions
  • Privacy policy
  • Contact us

Contact

18 Prowse Street
West Perth
WA 6005
P : +61 8 9200 5300
F : +61 8 9200 5324
E : [email protected]

  • Facebook
  • Instagram
  • Linkedin
  • Email

PBI is registered as an Income Tax Exempt Charity
CFN: CC21886
ACN: 164 941 815
ABN: 24 164 941 815

© Perth Blood Institute 2020