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haemophilia <disease, haematology> A haemorrhagic diathesis occurring in two main forms:
1. Haemophilia A (classic haemophilia, factor VIII deficiency), an X linked disorder due to deficiency of coagulation factor VIII.
2. Haemophilia B (factor IX deficiency, Christmas disease), also X linked, due to deficiency of coagulation factor IX.
Both forms are determined by a mutant gene near the telomere of the long arm of the X chromosome (Xq), but a different loci and are characterised by subcutaneous and intramuscular haemorrhages, bleeding from the mouth, gums, lips and tongue, haematuria and haemarthroses.
(18 Nov 1997)
haemophilia A <haematology> A sex-linked genetic disease affecting males that results from a deficiency of clotting factor VIII, a protein factor that is required for normal blood coagulation.
Symptoms include easy bruising, bleeding gums, nosebleeds and bleeding into muscle tissue (haematoma) or a joint space (haemarthrosis).
Treatment includes the infusion of factor VIII concentrate intravenously to restore this essential factor and normalize blood coagulation.
Inheritance: sex-linked.
(27 Sep 1997)
haemophilia B <haematology> A form of haemophilia in males that results from a deficiency of clotting factor IX, transmitted as a X-linked trait.
Symptoms include easy bruising, nosebleeds, bleeding gums and bleeding into muscle tissue (haematoma) or a joint space (haemarthrosis).
Treatment includes the infusion of factor IX concentrates to normalize blood coagulation.
Inheritance: sex-linked.
(27 Sep 1997)
haemophilia C <haematology> A patient afflicted with a sex-linked genetic disease affecting males that results from a deficiency of a specific protein factor that is required for normal blood coagulation.
See: haemophilia A and haemophilia B.
(27 Sep 1997)
Previous: haemophagocytosis, haemophil, haemophilia, haemophilia A, haemophilia BNext: haemophilia C, haemophilic, haemophilic arthritis, haemophilic bacteriahaemophilia C
<haematology> Haemophilia due to deficiency of factor XI; clinically resembles haemophilia A and B but has different inheritance.
Inheritance: autosomal dominant.
(20 Sep 2002)
haemophiliac <haematology> A patient afflicted with a sex-linked genetic disease affecting males that results from a deficiency of a specific protein factor that is required for normal blood coagulation.
See: haemophilia A and haemophilia B.
(27 Sep 1997)
haemophilic Relating to haemophilia.
(05 Mar 2000)
haemophilic arthritis <orthopaedics, rheumatology> Joint disease resulting from haemophilic bleeding into a joint.
(05 Mar 2000)
haemophilic bacteria <microbiology> Haemophilic bacteria are bacteria which thrive in the presence of blood.
These bacteria all belong to the taxonomic genera of Haemophilus, Bordetella, and Moraxella. They are rod-shaped, gram-negative, nonmotile parasites.
(09 Oct 1997)
haemophilic joint <orthopaedics> Chronic arthroplasty due to repeated haemarthrosis in a haemophiliac.
(05 Mar 2000)
haemophilioid <haematology> Haemophilioid refers to any disease which resembles haemophilia but is not the sole result of abnormal blood clotting. These diseases can be inherited or acquired.
(09 Oct 1997)
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