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V Deficiency (v + deficiency)

Distribution by Scientific Domains
Medical Sciences100%

Kinds of V Deficiency

  • factor v deficiency
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    Selected Abstracts

    Factor V deficiency: a concise review

    HAEMOPHILIA, Issue 6 2008
    J. N. HUANG
    Summary., Factor V (FV; proaccelerin or labile factor) is the plasma cofactor for the prothrombinase complex that activates prothrombin to thrombin. FV deficiency can be caused by mutations in the FV gene or in genes encoding components of a putative cargo receptor that transports FV (and factor VIII) from the endoplasmic reticulum to the Golgi. Because FV is present in platelet ,-granules as well as in plasma, low FV levels are also seen in disorders of platelet granules. Additionally, acquired FV deficiencies can occur in the setting of rheumatologic disorders, malignancies, and antibiotic use and, most frequently, with the use of topical bovine thrombin. FV levels have limited correlation with the risk of bleeding, but overall, FV-deficient patients appear to have a less severe phenotype than patients with haemophilia A or B. The most commonly reported symptoms are bleeding from mucosal surfaces and postoperative haemorrhage. However, haemarthroses and intramuscular and intracranial haemorrhages can also occur. Because no FV-specific concentrate is available, fresh frozen plasma remains the mainstay of treatment. Antifibrinolytics can also provide benefit, especially for mucosal bleeding. In refractory cases, or for patients with inhibitors, prothrombin complex concentrates, recombinant activated FVIIa, and platelet transfusions have been successfully used. Some patients with inhibitors may also require immunosuppression. [source]

    Urgent arterial: venous fistula to secure vascular access in a patient with severe factor V deficiency and intracranial haemorrhage

    HAEMOPHILIA, Issue 4 2007
    L. PEAKE
    No abstract is available for this article. [source]

    Severe factor V deficiency and pregnancy , a role for solvent,detergent plasma?

    HAEMOPHILIA, Issue 4 2005
    J. S. O'Donnell
    No abstract is available for this article. [source]

    Pulmonary embolism in a patient with severe congenital deficiency for factor V during treatment with fresh frozen plasma

    HAEMOPHILIA, Issue 3 2005
    A. García-Noblejas
    Summary., Thrombosis is a rare complication in patients with congenital clotting factor deficiencies. In most cases, it is related to inherited procoagulant factors, use of central venous catheters or administration of coagulation factor concentrates. There are only a few case reports about thrombotic events during treatment with fresh frozen plasma (FFP). We report the case of a patient with homozygous inherited factor V deficiency, who developed a pulmonary embolism at a time of treatment with methylene blue treated FFP (MBFFP). The patient had only two other factors predisposing to thrombosis and both were acquired: obesity and bed rest. He started anticoagulant treatment with low molecular weight heparin (LMWH) while the deficient factors were replaced with MBFFP. After 8 days of treatment the patient developed a severe respiratory insufficiency. Pulmonary haemorrhage was considered among the differential diagnosis and LMWH was stopped. An inferior vena cava filter was placed without any further thrombotic complications. To our knowledge, there are no reports about patients with clotting factor deficiencies who developed a thrombotic event during treatment with MBFFP. [source]

    Characterization of an immunologic polymorphism (D79H) in the heavy chain of factor V

    JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 6 2004
    M. Van Der Neut Kolfschoten
    Summary.,Background: During the study of a family with hereditary factor (F)V deficiency (FV Amersfoort, 1102 A > T in exon 7) we identified an individual with 5% FV heavy chain antigen (FVHC) and 50% FV light chain antigen (FVLC). Further testing revealed that apart from the FV Amersfoort allele a second variant FV allele was segregating in this family, which encodes for a FV molecule with a reduced affinity for mAb V-23 used in the FV heavy chain ELISA (ELISAHC). Objective: Identification and characterization of the molecular basis responsible for the reduced affinity of the variant FV for mAb V-23. Methods: Family members of the proband were screened for mutations in the exons coding for the heavy chain of FV, after which the recombinant variant FV could be generated and characterized. Next, the cases and controls of the Leiden Thrombophilia Study (LETS) were genotyped for carriership of the variant FV. Results: In the variant FV allele a polymorphism in exon 3 (409G > C) was identified, which predicts the replacement of aspartic acid 79 by histidin (D79H). Introduction of this mutation in recombinant FV confirmed that it reduces the affinity for binding to mAb V-23. The substitution has no effect on FV(a) stability and Xa-cofactor activity. In Caucasians the frequency of the FV-79H allele is ,5%. Analysis of the LETS revealed that the FV-79H allele is not associated with FV levels (FVLC), activated protein C sensitivity (using an activated partial thromboplastin time-based test) or risk of venous thrombosis (OR 1.07, CI 95: 0.7,1.7). Conclusion: The D79H substitution in FV should be considered as a neutral polymorphism. The monoclonal antibody V-23, which has a strongly reduced affinity for FV-79H, is not suitable for application in diagnostic tests. [source]

    Recurrent transfusion-related acute lung injury after fresh frozen plasma in a patient with hereditary factor V deficiency

    AMERICAN JOURNAL OF HEMATOLOGY, Issue 8 2008
    Alvaro Laga
    No abstract is available for this article. [source]