Details

Autor(en) / Beteiligte

• PEYVANDI, FLORA
• BOLTON-MAGGS, PAULA HB
• BATOROVA, ANGELIKA
• DE MOERLOOSE, PHILIPPE

Titel

Rare bleeding disorders

Ist Teil von

• Haemophilia : the official journal of the World Federation of Hemophilia, 2012-07, Vol.18 (s4), p.148-153

Ort / Verlag

Oxford, UK: Blackwell Publishing Ltd

Erscheinungsjahr

2012

Link zum Volltext

Quelle

Wiley Online Library Journals Frontfile Complete

Beschreibungen/Notizen

• Rare bleeding disorders (RBDs) include the inherited deficiencies of fibrinogen, factor (F)II, FV, FV+FVIII, FVII, FX, FXI and FXIII. There have been remarkable advances in understanding the molecular profiles that lead to each type of coagulation factor deficiency. However, as a consequence of their rarity, clinical data regarding the characteristics of bleeding symptoms and their management remain limited. The clinical manifestations in different RBDs are heterogeneous, and the residual plasma coagulant factor level does not always predict bleeding tendency. In this review, we describe the general features and recent advances in understanding three such deficiencies: FXI, FVII and fibrinogen deficiencies.