Treatment Guidelines

Haemophilia in South Africa

These guidelines have been compiled by the South African Haemophilia Foundation Medical and Scientific Advisory Council (SAHF MASAC) to facilitate the appropriate management of people with haemophilia (PWH). The current guidelines are based on the publication noted below.

Mahlangu J, Gillham A; South African Medical Journal, February 2008, 98(2):127 – 138

This guide is also available to be downloaded in .pdf format here.

This guide is intended for healthcare personnel who might not be familiar with Haemophilia. People with Haemophilia (PWH) and their physicians should be advised by a comprehensive Haemophilia Treatment Centre staffed by a multidisciplinary team skilled in the care of this uncommon chronic bleeding disorder.

Parents of patients with severe Haemophilia are usually trained in home infusion of the clotting Factor when their child is about four years old and self infusion is normally accomplished by 12 – 14 years of age. However, infants and boys with mild Haemophilia must rely on a Haemophilia Centre or other medical facility for clotting Factor infusions.

Click here to contact your nearest Haemophilia Treatment Centre if you have any uncertainty regarding management.

Acknowledgement is made of all the past and current members of MASAC, who have produced the previous edition, and reviewed the current edition.


Haemophilia is classified as severe, moderate, or mild according to the levels of circulating Factor VIII or IX and indicates the expected frequency of bleeding:


Factor VIII or IX < 1%

Factor VIII or IX replacement is needed several times per month for traumatic or apparently spontaneous bleeding may be on regular prophylactic factor therapy.


Factor VIII or IX 1 – 5%

Less frequent bleeding which usually follows trauma, surgery or dental work.


Factor VIII or IX > 5 – < 40%

Occasional bleeding, usually only after severe trauma or surgery.

Factor VIII Inhibitors in Haemophilia

Inhibitors may develop in 10 – 15% of persons with haemophilia A but are much less common in haemophilia B (1 – 3%). Inhibitors are neutralising antibodies that limit the effectiveness of Factor infusions.

Risk Factors for the development of inhibitors:
severe haemophilia
family history of inhibitor development
more frequent in black patients

If an individual is going to develop an inhibitor, this usually happens within the first 50 exposure days after starting Factor VIII replacement therapy.

Inhibitors titres are measured in Bethesda units (BU)
Low Responders:< titre remains below < 5 BU
High Responders: titre above > 5 BU. The level may increase markedly and rapidly after Factor VIII infusion (may have rapid anamnestic response in 3 days)

Rules for Inhibitor Management

Monitor all patients every 3 - 6 months for the development of inhibitors. This is particularly important and should be done more frequently in newly diagnosed black patients with severe haemophilia A, who are at greater risk.
Never undertake a surgical procedure or joint aspiration in a person with Haemophilia without checking for inhibitors.
If there is no response to appropriate replacement therapy, test for inhibitors.

Go to the next sections in Treatment Guidelines for Haemophilia