Rare bleeding disorders - bleeding assessment tools, laboratory aspects and phenotype and therapy of FXI deficiency

Rare bleeding disorders: genetic, laboratory, clinical, and molecular aspects. Preface.

Welcome to this special issue of Seminars in Thrombosis & Hemostasis. Characteristically, each issue of Seminars in Thrombosis & Hemostasis is theme driven, with each new issue devoted to a particular theme of relevance to thrombosis and hemostasis. The current issue of Seminars in Thrombosis & Hemostasis carries the theme of “Rare Bleeding Disorders” (RBDs) and is an update on a previous issue...

Rare Bleeding Disorders: Genetic, Laboratory, Clinical, and Molecular Aspects

Welcome to this special issue of Seminars in Thrombosis & Hemostasis. Characteristically, each issue of Seminars in Thrombosis & Hemostasis is theme driven, with each new issue devoted to a particular theme of relevance to thrombosis and hemostasis. The current issue of Seminars in Thrombosis & Hemostasis carries the theme of “Rare Bleeding Disorders” (RBDs) and is an update on a previous issue...

Rare bleeding disorders.

During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. Defects in platelets as well as inherited deficiencies of coagulation factors including fibrinogen...

Rare bleeding disorders: diagnosis and treatment.

Despite the worldwide prevalence of rare bleeding disorders (RBDs), knowledge of these conditions and their management is suboptimal; health care professionals often have little diagnostic and treatment experience with variable access to diagnostic modalities required for accurate identification. Therefore, patients often experience morbidity and mortality due to delayed diagnosis. As RBDs repr...

Factor XI Deficiency: a study of clinical, laboratory and molecular modifiers of bleeding phenotype