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Hemophilia is a bleeding disorder that slows down the blood clotting process. People who
have hemophilia often have longer bleeding after an injury or surgery. People who have
severe hemophilia have spontaneous bleeding into the joints and muscles. Hemophilia occurs
more commonly in males than in females.
TYPES OF HAEMOPHILIA:
The two most common types of hemophilia are hemophilia A (also known as classic
hemophilia) and hemophilia B (also known as Christmas disease). People who have
hemophilia A have low levels of a blood clotting factor called factor eight (FVIII). People
who have hemophilia B have low levels of factor nine (FIX). Hemophilia A is the most
common type of this condition. One in 5,000 to 10,000 males worldwide have hemophilia A.
Hemophilia B is less common, and it affects 1 in 20,000 to 34,500 males worldwide.
LEVELS OF SEVERITY:
There are 3 levels of severity in haemophilia: mild, moderate and severe. The level of
severity depends on the amount of clotting factor in the person's blood. A person with
haemophilia will have the same level of severity over their lifetime. Within a family, males
with haemophilia will also have the same level of severity. The normal range of factor VIII
and factor IX in a person's blood is between 50% and 150%.
MILD HAEMOPHILIA:
Present in 5-40 per cent of normal clotting factor
Usually only have bleeding problems after having teeth taken out, surgery or a bad
injury or accident.
Might never have a bleeding problem.
MODERATE HAEMOPHILIA:
Present in 1-5 per cent of normal clotting factor
Might have bleeding problems after having teeth taken out, surgery or a bad injury or
accident
May have bleeding problems after minor injuries such as sporting injuries
Rarely have a bleed for no obvious reason.
SEVERE HAEMOPHILIA:
Present in less than 1 per cent of normal clotting factor
Often have bleeds into joints, muscles and soft tissues
Can have bleeds for no obvious reason, as well as after surgery, dental work or
injuries including minor bumps or knocks
HAEMOPHILIA INHERITENCE:
Haemophilia is an inherited condition and occurs in families. Men with haemophilia will pass
the gene on to their daughters but not to their sons. Women who carry the haemophilia gene
can pass the haemophilia gene on to their sons and daughters. Sons with the gene will have
haemophilia.
TESTS AND DIAGNOSIS:
Hemophilia A and B are diagnosed by measuring factor clotting activity. Individuals
who have hemophilia A have low factor VIII clotting activity. Individuals who have
hemophilia B have low factor IX clotting activity.
Genetic testing is also available for the factor VIII gene and the factor IX gene.
Genetic testing of the FVIII gene finds a disease-causing mutation in up to 98 percent
of individuals who have hemophilia A. Genetic testing of the FIX gene finds disease-
causing mutations in more than 99 percent of individuals who have hemophilia B.
Genetic testing is usually used to identify women who are carriers of a FVIII or FIX
gene mutation, and to diagnose hemophilia in a fetus during a pregnancy (prenatal
diagnosis). It is sometimes used to diagnose individuals who have mild symptoms of
hemophilia A or B.