Familial multiple coagulation factor deficiencies: new biologic insight from rare genetic bleeding disorders

Familial multiple coagulation factor deficiencies: new biologic insight from rare genetic bleeding disorders.

Combined deficiency of factor (F)V and FVIII (F5F8D) and combined deficiency of vitamin K-dependent clotting factors (VKCFD) comprise the vast majority of reported cases of familial multiple coagulation factor deficiencies. Recently, significant progress has been made in understanding the molecular mechanisms underlying these disorders. F5F8D is caused by mutations in two different genes (LMAN1...

Multiple congenital coagulation deficiencies.

A 6-week-old infant is presented who suffered from a congenital haemorrhagic disorder which caused death from subdural haemorrhage following mild trauma. Haematological investigation revealed deficiencies of factor VII and Christmas factor. Prower-Stuart factor was probably also deficient although investigation of this clotting factor was carried out only on serum obtained at necropsy.

Postoperative bleeding and coagulation disorders.

PURPOSE OF REVIEW New data have been made available in the field of haemostasis and thrombosis. Some long-awaited answers to important questions have been published, and some debates have benefited from an updated perspective. RECENT FINDINGS Two important domains are addressed in this update: massive bleeding (1/1/1 ratios and fibrinogen concentrates) and the management of direct oral antico...

The Rare Coagulation Disorders

Epidemiology of Hereditary Coagulation Bleeding Disorders: A 15-Year Experience From Southern Iran

Background: Data on the frequency of hereditary bleeding disorders (HBDs) and associated mortality and morbidities during a long-term follow-up from Iran are scarce. Objective: This study evaluated the epidemiologic features among patients with HBD in one of the largest referral centers in southern Iran. Methods: In this cross-secti...