Background Congenital factor VII (FVII) deficiency is a rare autosomal recessive coagulation disorder that is characterized by prolongation of prothrombin time. Recombinant activated FVII (rFVIIa) is widely used in the management of bleeding in patients with congenital FVII deficiency. We experienced anesthetic management of a patient with congenital FVII deficiency who was scheduled for laparo...

Thrombophilic disorders encompass a diverse group of coagulation disorders that potentiate a predisposition for thrombotic events (e.g. deep vein thrombosis and pulmonary embolism). The term thrombophilia was coined in 1965 following a Norwegian familial study of venous thrombosis [1]. The entity of thrombophilia has also been described as a disorder in which there is abnormally, enhanced coagu...

In Western countries, venous thromboembolism (VTE) is a widespread and serious disorder, with hospital admission rates that appear to be increasing. Current anticoagulant therapies available for the prevention and treatment of VTE have several drawbacks that make them either difficult to manage effectively, due to a need for careful monitoring to control coagulation, or, in the case of parenter...

Von Willebrand's disease is the most common hereditary coagulation disorder in young women. This report aimed at describing the case of obese patient with von Willebrand's disease scheduled for cesarean section. We report the case of an obese patient, 26 years old, with von Willebrand's disease, admitted to the emergency room in early labor. Cesarean section was indicated because the baby shows...

Factor IX deficiency was associated with a hemorrhagic disorder in a woman who previously had experienced postpartum hypotension resulting in Sheehan’s syndrome. There was no family history of Christmas disease, and other known causes of factor IX deficiency were excluded. Plasma levels of factor IX were partially corrected by therapy with thyroid hormones and completely restored to normal by t...

© 2009 The Authors. doi: 10.2340/00015555-0733 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Purpura fulminans (PF) is a rare disorder characterized by thrombosis of dermal vessels and skin necrosis with or without consumptive coagulopathy (1). PF is usually classified into three types: haemostasis-initiated PF or neonatal PF; acute infectious PF; and idiopathic PF ...

Instead of pulsatile ventricular assist devices an increasing number of nonpulsatile ventricular assist devices are introduced to clinical practice. The different flow characteristics of this new technique lead to alteration in shear stress on blood components, which may affect the coagulation system. Repeated von Willebrand factor analyses were performed in a patient who first was implanted wi...

Adult onset Still's disease is uncommon in middle-aged and elderly individuals and can rarely present with shock; shock is usually associated with disseminated intravascular coagulation, multiorgan dysfunction syndrome or acute respiratory distress syndrome. We report a post-menopausal woman with arthritis, fever, pneumonitis and hypotension which was managed as septic shock. Steroids were inad...

In a report reading like a fascinating detective story, Vincent and colleagues crack the mysterious case of east Texas bleeding disorder. They show that affected individuals have a mutation in exon 13 of the coagulation F5 gene that causes increased expression of an alternatively spliced transcript, which encodes a previously unrecognized factor V (FV) isoform they call FV-short. This FV isofor...

Membrane-bound Factor VIII (FVIII) has a critical function in blood coagulation as the pro-cofactor to the serine-protease Factor IXa (FIXa) in the FVIIIa-FIXa complex assembled on the activated platelet membrane. Defects or deficiency of FVIII cause Hemophilia A, a mild to severe bleeding disorder. Despite existing crystal structures for FVIII, its membrane-bound organization has not been reso...