haemophilia(redirected from Hæmophilia)
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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.
[hemo- + G. philos, fond]
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
haemophiliaAn 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.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
haemophiliaa 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.
Collins Dictionary of Biology, 3rd ed. © W. G. Hale, V. A. Saunders, J. P. Margham 2005
Inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhages, spontaneous or traumatic, because of a defect in the blood-coagulating mechanism.
[hemo- + G. philos, fond]
Medical Dictionary for the Dental Professions © Farlex 2012