Guideline for the diagnosis and management of the rare coagulation disorders.

AD Mumford, S Ackroyd, R Alikhan… - British journal of …, 2014 - search.ebscohost.com
The article discusses the guidelines of the United Kingdom Haemophilia Centre Doctors'
Organization (UKHCDO) related to the diagnosis and management of patients with rare …

Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders

F Peyvandi, R Palla, M Menegatti… - … of Thrombosis and …, 2012 - Wiley Online Library
Background: The European Network of Rare Bleeding Disorders (EN‐RBD) was established
to bridge the gap between knowledge and practise in the care of patients with RBDs …

Combined deficiency of coagulation factors V and VIII: an update

C Zheng, B Zhang - Seminars in thrombosis and hemostasis, 2013 - thieme-connect.com
Combined deficiency of factor V (FV) and FVIII (F5F8D) is an autosomal recessive bleeding
disorder characterized by simultaneous decreases of both coagulation factors. This review …

Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran

A Dorgalaleh, SER Alavi, S Tabibian, S Soori… - …, 2017 - Taylor & Francis
ABSTRACT Background: Rare bleeding disorders (RBDs) are heterogeneous disorders,
mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate …

Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2

B Zhang, B McGee, JS Yamaoka, H Guglielmone… - Blood, 2006 - ashpublications.org
Mutations in LMAN1 (ERGIC-53) or MCFD2 cause combined deficiency of factor V and
factor VIII (F5F8D). LMAN1 and MCFD2 form a protein complex that functions as a cargo …

The use of prophylaxis in the treatment of rare bleeding disorders

A Shapiro - Thrombosis Research, 2020 - Elsevier
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation factor
deficiencies that include fibrinogen, prothrombin, α 2-antiplasmin, plasminogen activator …

Management of rare inherited bleeding disorders: proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

M Trossaert, V Chamouard… - European Journal of …, 2023 - Wiley Online Library
Introduction The rare coagulation disorders may present significant difficulties in diagnosis
and management. In addition, considerable inter‐individual variation in bleeding phenotype …

Combined FV and FVIII deficiency

M Spreafico, F Peyvandi - Haemophilia, 2008 - Wiley Online Library
Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to
lifelong bleeding disorders. The severity of these disorders is generally inversely …

Recent developments in the understanding of the combined deficiency of FV and FVIII

B Zhang - British journal of haematology, 2009 - Wiley Online Library
Combined deficiency of factor V (FV) and factor VIII (FVIII)(F5F8D) is a genetic disorder
characterized by mild‐to‐moderate bleeding and coordinate reduction in plasma FV and …

Low factor V level ameliorates bleeding diathesis in patients with combined deficiency of factor V and factor VIII

Y Shao, W Wu, G Xu, X Wang… - Blood, The Journal of the …, 2019 - ashpublications.org
Combined factor V (FV) and FVIII deficiency (F5F8D) is a rare autosomal-recessive bleeding
disorder caused by mutations in lectin mannose binding-1 (LMAN1) and multiple …