Genotype-phenotype correlation in combined deficiency of factor V and factor VIII

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FACTOR V AND VIII INHIBITOR IN PATIENTS WITH COMBINED FACTOR V AND VIII DEFICIENCY

Patients with coagulation factor(s) deficiency who use coagulation therapy are susceptible to forming inhibitors against coagulation factor(s). In this survey we detected factor V and VIII inhibitor in ten patients with combined deficiency of factors V and VIII from north east of Iran (Khorassan province). It was revealed in our survey that eight patients had both factor V and factor VIII i...

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Combined Factor V and VIII Deficiency

This review summarizes current data on the pathomechanisms and new genetic findings of combined factor V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined factor V and VIII deficiency (F5F8D) was reported b...

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Genotype-phenotype correlation in combined deficiency of factor V and factor VIII.

Combined deficiency of factor V and factor VIII (F5F8D) is caused by mutations in one of 2 genes, either LMAN1 or MCFD2. Here we report the identification of mutations for 11 additional F5F8D families, including 4 novel mutations, 2 in MCFD2 and 2 in LMAN1. We show that a novel MCFD2 missense mutation identified here (D81Y) and 2 previously reported mutations (D89A and D122V) abolish MCFD2 bind...

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factor v and viii inhibitor in patients with combined factor v and viii deficiency

patients with coagulation factor(s) deficiency who use coagulation therapy are susceptible to forming inhibitors against coagulation factor(s). in this survey we detected factor v and viii inhibitor in ten patients with combined deficiency of factors v and viii from north east of iran (khorassan province). it was revealed in our survey that eight patients had both factor v and factor viii inhib...

متن کامل

combined factor v and viii deficiency

this review summarizes current data on the pathomechanisms and new genetic findings of combined factor v and viii deficiency (cf5f8d). congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. among dual coagulation disorders, cf5f8d is the most common type. for the first time combined factor v and viii deficiency (f5f8d) was reported b...

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ژورنال

عنوان ژورنال: Blood

سال: 2008

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood-2007-10-113951