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definition |
| Rare
bleeding disorders (RBDs) are very rare diseases, . Haemophilia
A and B are the most frequent inherited bleeding disorders.
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| Together
with von Willebrand disease, a defect of primary hemostasis
associated with a secondary defect in coagulation factor VIII
(FVIII), these X-linked disorders include 95% to 97% of all
the inherited deficiencies of coagulation factors -1-.
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| The
other 3-5% are represented by the less common inherited disorders.
This include deficiency of fibrinogen, prothrombin, factors
V, combined V+VIII, VII, X, XI and XIII. |
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| These
disorders are inherited in an autosomal recessive manner (Figure
1) and their prevalence is approximately 1:500.000 or less in
the general population of western countries |
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| However
their frequency is increased 10-20 times where consanguineous
marriages are practised such as Middle East countries and in
Southern India - 2-. |
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| The natural history and spectrum of clinical manifestations of RBDs are not well established, since few centres in the world have the opportunity to see a significant number of these rare patients. |
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