[HTML][HTML] Hemophilia therapy: the future has begun

PM Mannucci - Haematologica, 2020 - ncbi.nlm.nih.gov
The success story of hemophilia care first began in the 1970s, when the availability of
plasma-derived concentrates of coagulation factor VIII (FVIII) and factor IX (FIX) provided …

Blood coagulation in immunothrombosis—at the frontline of intravascular immunity

F Gaertner, S Massberg - Seminars in immunology, 2016 - Elsevier
While hemostasis is the physiological process that prevents blood loss after vessel injury,
thrombosis is often portrayed as a pathologic event involving blood coagulation and platelet …

Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology: first update 2016

SA Kozek-Langenecker, AB Ahmed… - European Journal of …, 2017 - journals.lww.com
The management of perioperative bleeding involves multiple assessments and strategies to
ensure appropriate patient care. Initially, it is important to identify those patients with an …

Treatment of rare factor deficiencies other than hemophilia

M Menegatti, F Peyvandi - Blood, The Journal of the American …, 2019 - ashpublications.org
The deficiency of fibrinogen, prothrombin, factor V (FV), FVII, FVIII, FIX, FX, FXI, and FXIII,
called rare coagulation disorders (RCDs), may result in coagulopathies leading to …

[HTML][HTML] Factor XI deficiency

MD Lewandowska, JM Connors - Hematology/Oncology …, 2021 - hemonc.theclinics.com
Factor XI (FXI) deficiency (hemophilia C or Rosenthal disease) was first described in the
1950s by Rosenthal and colleagues 1 in four generations of a family experiencing bleeding …

[HTML][HTML] Diagnosis and classification of congenital fibrinogen disorders: communication from the SSC of the ISTH

A Casini, A Undas, R Palla, J Thachil… - Journal of Thrombosis …, 2018 - Elsevier
Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare inherited
abnormalities of blood coagulation 1. CFDs result from monoallelic or biallelic mutations in …

[HTML][HTML] Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management

G Benson, G Auerswald, G Dolan, A Duffy… - Blood …, 2018 - ncbi.nlm.nih.gov
Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is
characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it …

[HTML][HTML] Biochemical, molecular and clinical aspects of coagulation factor VII and its role in hemostasis and thrombosis

F Bernardi, G Mariani - haematologica, 2021 - ncbi.nlm.nih.gov
Activated factor VII (FVIIa), the first protease of clotting, expresses its physiological
procoagulant potential only after complexing with tissue factor (TF) exposed to blood. Deep …

Clinical features and management of congenital fibrinogen deficiencies

A Casini, P de Moerloose… - Seminars in thrombosis …, 2016 - thieme-connect.com
Congenital fibrinogen disorders are rare diseases affecting either the quantity
(afibrinogenemia and hypofibrinogenemia) or the quality (dysfibrinogenemia) or both …

[HTML][HTML] Hemostatic challenges in neonates

P Davenport, M Sola-Visner - Frontiers in pediatrics, 2021 - frontiersin.org
The neonatal hemostatic system is strikingly different from that of adults. Among other
differences, neonates exhibit hyporeactive platelets and decreased levels of coagulation …