Blood Transfusion Supplement 4 - Plasma-derived medicinal products: demand and clinical use (off subscription) (September)
Present and future challanges in the treatment of haemophilia: a clinician's perspective
 
Authors:  Pier Mannuccio Mannucci, Massimo Franchini
Pages:  77-81
To cite this article:  Blood Transfus 2013; 11 Suppl 4: s77-81
Doi:  10.2450/2013.012s
Published online:  08/10/2013

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Introduction
Haemophilia A and B are rare bleeding disorders caused by mutations in the genes encoding coagulation factor VIII (FVIII) and factor IX (FIX)1. The prevalence of haemophilia A is 1 in 5,000 males, that of haemophilia B is 1 in 40,0001,2. Patients with plasma factor levels <1 IU/dL are classified as having severe haemophilia, those with levels between 1-5 IU/dL and >5 IU/dL are classified as having moderate or mild haemophilia3. Patients with mild haemophilia bleed excessively only in response to surgery, tooth  extractions or major injuries, whereas patients with moderate haemophilia bleed excessively after relatively minor trauma and those with severe haemophilia bleed spontaneously or after trivial trauma. Severe haemophilia is epitomised by limb- or life-threatening symptoms such as haemarthrosis, soft-tissue haematoma, retroperitoneal and intracerebral haemorrhage and post-surgical bleeds. (...)
  
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