haemophilia

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haemophilia

he·mo·phil·i·a

(hē'mō-fil'ē-ă)
An inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhages, spontaneous or traumatic, due to a defect in the blood coagulating mechanism.
Synonym(s): haemophilia.
[hemo- + G. philos, fond]

haemophilia

An X-linked recessive blood clotting disorder causing a life-long tendency to excessive bleeding. It cannot be passed from father to son because the father transmits only the Y chromosome to his sons. All the daughters of a haemophilic man are carriers of the gene but do not suffer the disease. There is a 50% chance that each of their sons will be a haemophiliac. Females can acquire the disease only if both X chromosomes carry the gene. Haemophilia A is due to the absence of Factor VIII, one of the coagulation factors. Haemophilia B (Christmas disease) is due to deficiency of Factor IX. Both feature bleeding, either spontaneous or on minor trauma, most commonly into the joints. This causes severe pain, swelling and muscle spasm. Repeated episodes lead to damage and severe joint disability. Tooth extraction or external injury are followed by prolonged bleeding. Spontaneous bleeding may occur into the bowel. Haemophilia is treated by repeated injections of Factor VIII or IX obtained from donated blood.

haemophilia

a rare human blood disorder in which BLOOD CLOTTING is deficient, resulting often in severe bleeding internally and externally. The condition is due to a lack of fibrin in the blood and is controlled by two closely linked genes on the X-CHROMOSOME that are responsible for the production of different clotting factors. Haemophilia A individuals lack antihaemophilic globulin (AHG) while haemophilia B individuals lack plasma thromboplastin. Males carrying the mutant ALLELE of either locus or (much more rarely) females homozygous for the recessive mutant alleles of either locus will be affected, although heterozygous females have normal blood. Haemophilia A is by far the most common form of the disease (about 80%) and can be treated by transfusions of AHG.

haemophilia

inherited coagulation cascade disorder, characterized by lifelong tendency to haemorrhage
  • haemophilia A; HA deficiency of factor VIII; X-linked recessive condition, expressed almost exclusively in males; characterized by delayed clotting (decreased formation of thromboplastin and reduced conversion of prothrombin)

  • haemophilia B; HB; Christmas disease see disease, Christmas

  • vascular haemophilia; von Willebrand's disease see disease, von Willebrand's

he·mo·phil·i·a

(hē'mō-fil'ē-ă)
Inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhages, spontaneous or traumatic, because of a defect in the blood-coagulating mechanism.
Synonym(s): haemophilia.
[hemo- + G. philos, fond]
References in periodicals archive ?
The Irish Haemophilia Society had thought that 221 haemophiliacs were infected.
He is only 46 but has been to 97 funerals of haemophiliacs who have died of Aids - the result of taking the very blood products they depend on to maintain a normal day-to-day life.
Nearly 2,000 haemophiliacs have died since they were exposed to the fatal viruses in contaminated blood or blood products more than 20 years ago and many others are said to be terminally ill.
The therapy is normally used to treat haemophiliacs whose blood does not clot properly.
Now 500 haemophiliacs in Scotland are being urged to get check-ups to see if they received a batch of infected blood.
English and Northern Irish sufferers from the illness, which prevents blood clotting, are denied access to an artificial treatment which is available to haemophiliacs in Scotland and Wales.
During the 1970s and 1980s, some 95pc of Britain's haemophiliacs contracted hepatitis C and 25pc HIV as a result of treatment with contaminated blood products.
But campaigners, including widow Carol Grayson, of Jesmond, Newcastle, who has fought for years for an open inquiry into how her haemophiliac husband Peter Longstaff was infected with both HIV and Hepatitis C, are hoping the new independent inquiry will finally provide them with answers.
Mr Norval called for a review of the treatment of haemophiliacs.
Mr Carruthers, 54, of Horsley Road, Barmston, Washington, is one of more than 4,000 haemophiliacs who developed hepatitis C or HIV in the 1970s and 80s after being given imported blood products supplied by paid donors in the US and South Africa.
It was only years later, as haemophiliacs were being diagnosed with HIV in the 1980s, that Lord Owen realised his plans had not been carried out.
1960s ( First blood transfusions carried out on haemophiliacs in Britain.