Work

Rare bleeding disorders

Year: 2012

Type: article

Abstract: Summary. 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.

Source: Haemophilia

DOI: 10.1111/j.1365-2516.2012.02841.x

Authors: FLORA PEYVANDI, PAULA HB BOLTON‐MAGGS, ANGELIKA BATOROVA, PHILIPPE DE MOERLOOSE

Institutions: University of Milan, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Manchester, University Hospital Bratislava, Comenius University Bratislava, University of Geneva, University Hospital of Geneva

Language: English

Topic: Hemophilia Treatment and Research

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W2320324893

FWCI: 4.615

Cites: 51

Cited by: 80

Related to: 10