Factor V Deficiency

A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren's disease or parahemophilia. It varies greatly in severity. Factor V deficiency is an autosomal recessive trait. (Dorland, 27th ed)

Also Known As:

Networked: 151 relevant articles (2 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemorrhage
2.	Activated Protein C Resistance (APC Resistance)
3.	Hemophilia A (Haemophilia)
4.	Antiphospholipid Syndrome (Antiphospholipid Antibody Syndrome)
5.	Protein C Deficiency

Experts

1.	Tenchini, M L: 4 articles (06/2003 - 12/2000)
2.	Montefusco, M C: 4 articles (06/2003 - 12/2000)
3.	Asselta, R: 4 articles (06/2003 - 12/2000)
4.	Duga, S: 4 articles (06/2003 - 12/2000)
5.	Radu, Claudia: 3 articles (11/2013 - 11/2008)
6.	Simioni, Paolo: 3 articles (11/2013 - 11/2008)
7.	Castoldi, Elisabetta: 3 articles (11/2013 - 11/2008)
8.	Spiezia, Luca: 3 articles (11/2013 - 11/2008)
9.	Castoldi, E: 3 articles (05/2011 - 06/2001)
10.	Asselta, Rosanna: 3 articles (03/2009 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Factor V Deficiency:

1.	Factor V (Coagulation Factor V)IBA 12/20/1993 - "Factor V deficiency and specific inhibitors of factor V yielded, in some instances, false positive results." 10/01/2008 - "In the present study, we investigated the molecular basis of factor V deficiency in three patients, and performed a comprehensive analysis of the factor V gene (F5) splicing pattern. " 01/01/1996 - "These studies provide the first evidence of a genetic component in determining factor V levels in plasma and of a genetic linkage between the factor V gene and factor V deficiency. " 07/01/1955 - "Hereditary deficiency of proaccelerin (parahemophilia): a family study." 04/01/2014 - "Recently, we detected two genetic variations in factor V of a Chinese patient with hereditary factor V deficiency. "
2.	recombinant FVIIa (rFVIIa)IBA 03/01/2008 - "We conclude that massive cecal mucosal bleeding is a possible manifestation of homozygous factor V deficiency and rFVIIa could be a successful therapy." 01/01/2015 - "We describe a complicated case of an elderly patient with severe factor V deficiency with high inhibitor titer refractory to FEIBA (anti-inhibitor coagulation complex) treated with NovoSeven concurrently with cyclosporine immunosuppression and Rituxan. "
3.	Oral ContraceptivesIBA 01/01/2005 - "Pregnancy and oral contraceptives in factor V deficiency: a study of 22 patients (five homozygotes and 17 heterozygotes) and review of the literature." 09/01/2002 - "C and S deficiency, factor V Leiden, oral contraceptives). " 02/01/2015 - "Patient 1 is a 16-year-old female with Factor V deficiency and menorrhagia which was previously controlled with oral contraceptives (OC). " 01/01/2008 - "Control of ovulation-induced hemoperitoneum by oral contraceptives in a patient with congenital hypoprothrombinemia and in another with congenital factor V deficiency." 04/01/2009 - "Thrombin activity is raised in hypercoagulable states, such as antithrombin deficiency, protein C and S deficiency, factor V Leiden and in women under oral contraceptives. "
4.	factor V clotting antigenIBA 01/01/1996 - "An increasing frequency of the Arg1299 (R2 allele) correlated with a decreasing mean plasma factor V activity in the groups of subjects under study, which included 26 unrelated subjects with partial factor V deficiency. " 08/01/1983 - "The presence of patients with antigen similar in concentration to coagulant activity and antigen in excess of Factor V activity indicates the heterogeneity of congenital Factor V deficiency." 09/01/1999 - "Each of his two children inherited a different paternal factor V allele; a daughter was heterozygous for factor V Leiden, with 100% factor V activity, and a son was heterozygous for factor V deficiency, with 50% factor V activity. " 10/01/1978 - "Immunoreactive Factor V determined with an homologous antibody corresponded to the functional Factor V activity of platelets from one patient with Factor V deficiency, suggesting that the patient's platelets have a decreased amount of normal Factor V. The ability of platelets from the patients to bind Factor X(a) and increase the rate of thrombin formation correlated with the severity of each patient's bleeding disorder better than the plasma level of Factor V. The results indicate that Factor V is required for the Factor X(a)-platelet interaction and that thrombin formation at the platelet surface is important in normal hemostasis."
5.	Carrier Proteins (Binding Protein)IBA 06/15/1996 - "Recent studies identified multimerin as a specific coagulation factor V binding protein, complexed with platelet, but not plasma, factor V. These findings led us to investigate individuals with inherited factor V deficiencies for possible multimerin abnormalities. "
6.	multimerinIBA 06/15/1996 - "Recent studies identified multimerin as a specific coagulation factor V binding protein, complexed with platelet, but not plasma, factor V. These findings led us to investigate individuals with inherited factor V deficiencies for possible multimerin abnormalities. "
7.	Prothrombin (Factor II)IBA 02/01/1980 - "In blood from two patients with congenital Factor V deficiency, prothrombin activation during spontaneous in vitro clotting was delayed and decreased. " 04/30/2004 - "- Coagulation disorders, namely thrombophilia, are a frequent cause of RSA and probably RIF as well, the most relevant being antithrombin deficiency, Factor V Leiden and prothrombin mutations. " 01/10/2000 - "Patients and controls were considered thrombophilic when they had protein C deficiency, protein S deficiency, antithrombin deficiency, factor V Leiden mutation, or prothrombin 20210 A mutation. " 01/01/2008 - "Patients showed a higher significant percentage of ACAs titres IgG>41 GPL u/ml and LA than controls (P=0.009; P<0.001, respectively), although no differences in AT, PC, PS deficiencies, factor V Leiden and prothrombin G20210A mutation was observed (P>0.05). " 09/01/2007 - "There were no significant associations between recurrence of VTE and laboratory risk factors such as natural inhibitor deficiency, factor V Leiden, the G20210A prothrombin variation, elevated factor VIII or hyperhomocysteinemia. "
8.	Protein CIBA 12/01/2010 - "Whereas major risk factors in adults are the procoagulant states such as protein C or S deficiency, factor V Leiden mutation, primary or secondary antiphospholipid syndrome, severe hypothyroidism, and trauma. " 05/01/2000 - "31% of the patients had thrombotic risk factors (protein S, protein C, antithrombine III deficiency, factor V Leiden, etc). " 03/01/2005 - "Congenital hypercoagulable disorders are more prevalent in families or patients with a history of thromboembolic events early in life or multiple miscarriages and include conditions such as protein C or S deficiencies, factor V Leiden mutation, and prothrombin gene variant. " 11/01/1980 - "Protein C inhibitory activity is not detectable in plasmas from four unrelated patients with combined Factor V/VIII deficiency but is present in normal amounts in plasmas from patients with simple factor V deficiency or Factor VIII deficiency. " 08/01/2008 - "Recognized risk factors for this disease are the prothrombotic state of pregnancy (third trimester), excess weight, and thrombophilia Accidental dural puncture, protein C and S deficiencies, factor V Leiden mutation, antiphospholipid syndrome, and the use of oral contraceptives have also been implicated. "
9.	ThrombinFDA Link 05/01/2011 - "Homozygous F5 deep-intronic splicing mutation resulting in severe factor V deficiency and undetectable thrombin generation in platelet-rich plasma." 06/15/2003 - "To raise awareness of bovine thrombin-induced factor V deficiency. " 02/01/1993 - "Bovine thrombin-induced factor V deficiency may have been previously unrecognized. " 02/01/1993 - "Bovine thrombin-induced factor V deficiency was though to be a very rare acquired coagulopathy, with only three documented cases. " 03/01/2010 - "This report documents the first case of factor V deficiency and profound coagulopathy in a burn patient after intraoperative exposure to bovine thrombin. "
10.	Protein SIBA 05/01/2000 - "31% of the patients had thrombotic risk factors (protein S, protein C, antithrombine III deficiency, factor V Leiden, etc). " 07/01/2005 - "The HTRF studied were protein C (PC), protein S (PS) and antithrombin (AT) deficiencies, factor V Leiden (FVL) and factor II 20210A polymorphisms. " 07/01/2005 - "Commonly diagnosed thrombophilic states in our population currently include protein S or C deficiency, antiphospholipid antibodies, and less commonly ATIII deficiency, factor V Leiden mutation, G20210A PT mutation, and plasminogen activator inhibitor-1 mutation. " 08/01/1996 - "Factor V increased the rate of APC-mediated factor VIII inactivation in a dose-dependent manner in representative plasma samples with protein S or factor V deficiency, abnormal factor V (heterozygous or homozygous for factor VR506Q), or a combination of heterozygous protein S deficiency and heterozygous factor VR506Q. " 03/01/2000 - "The prothrombotic disorders that were investigated included classical and occult myeloproliferative disorders; antiphospholipid syndrome; protein C; protein S and antithrombin deficiency; factor V Leiden; factor II; and methylene-tetrahydrofolate-reductase gene mutations. "

Therapies and Procedures

1.	Bariatric Surgery 08/01/2006 - "Literature review indicates that this is the first reported case of bariatric surgery in a patient with factor V deficiency."
2.	Off-Pump Coronary Artery Bypass (Coronary Artery Bypass, Off Pump) 01/01/2005 - "We herein describe a patient with severe factor V deficiency who underwent successful off-pump coronary artery bypass grafting with a transfusion of fresh-frozen plasma." 01/01/2005 - "Off-pump coronary artery bypass grafting in a patient with congenital factor V deficiency: report of a case."
3.	Transplants (Transplant) 05/01/2005 - "We prospectively tested patients who were evaluated for a kidney transplant for 8 thrombophilias, protein S and C deficiencies, factor V Leiden mutation, antithrombin III deficiency, anticardiolipin antibody, lupus anticoagulant, prothrombin gene mutation, and heparin-induced platelet antibody (HIPA). "
4.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 10/01/1980 - "Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. "
5.	Extracorporeal Membrane Oxygenation (ECMO) 04/01/2015 - "This is the first reported case of acquired factor V deficiency in a patient receiving extracorporeal membrane oxygenation for Legionella pneumonia. "