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      Aortic Valve Replacement in Severe Factor V Deficiency and Inhibitor: Diagnostic and Management Challenges

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          Abstract

          Factor V (FV) deficiency (F5D) is a rare hematological disorder with a variable spectrum of bleeding manifestations. Until now, no specific protocols for the management of these patients have been established. However, available literature suggests that perioperative infusion of fresh frozen plasma (FFP) may help maintain FV levels to prevent bleeding. We present the case of a 64-year-old man with previously undiagnosed severe FV deficiency and mild FV inhibitor, who underwent aortic valve replacement with no bleeding complications.

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          The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study.

          F Rodeghiero, G Castaman, A Tosetto … (2005)
          The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and another first degree relative with type 1 VWD (affected subjects, AFF). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion). Sensitivity, specificity, diagnostic likelihood ratios, positive and negative predictive values for the diagnosis of type 1 VWD were calculated from the data collected in OC and in 215 controls. Having at least three hemorrhagic symptoms or a bleeding score of 3 in males and 5 in females was very specific (98.6%) for the bleeding history of type 1 VWD, although less sensitive (69.1%). None of the misclassified OC had life-threatening bleeding episodes after diagnosis. We suggest that the use of a standardized questionnaire and bleeding score may be useful for the identification of subjects requiring laboratory evaluation for VWD.
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            Acquired factor V inhibitor. A problem-based systematic review.

            Ai Ang, Ponnudurai Kuperan, Chin Ng … (2009)
            Acquired factor V(FV) inhibitors as a rare bleeding disorder, poses a formidable challenge to treating physicians with limited evidence to guide its management. We systematically reviewed our experience in Singapore and the published literature to determine possible answers to clinical questions formulated on the manifestation and best management of non-bovine thrombin and non-congenital acquired FV inhibitors. The incidence in Singapore was 0.09 cases per million person years (3 cases over 10 years). Seventy-three other cases meeting pre-defined search criteria were found in the published literature. Bleeding occurred in 68.4% of these patients, with mucous membranes being the most common site. Intracranial and retroperitoneal bleeds carried the highest mortality. The mortality rate from bleeding was 12%. There was a tendency for FV levels and PT/aPTT prolongation to predict bleeding but not the inhibitor level. No consistently effective haemostatic agent could be determined, but platelet transfusion should probably be the first line therapy. Among bleeding patients, inhibitors tended to disappear faster with inhibitor elimination therapy (IET) compared to without IET (60 vs. 150 days, p=0.299). IET made no significant difference among non-bleeding patients (p=0.511) and is thus recommended for bleeding patients or those with high bleeding risk. Steroids as single agent IET was effective in the majority of patients. Logical management approaches may be drawn but are limited by small sample size, heterogeneity of reports, and potential publication bias. The inception of a comprehensive registry will provide more reliable data that may verify our findings.
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              A new look at blood coagulation factor V.

              Rodney Camire (2011)
              Factor V plays an essential role in hemostasis and has a profound influence on thrombin generation. The aim of this review is to highlight recent advances in our understanding of the biology of factor V which shed light on the variable bleeding tendencies in severe factor V deficiency. Furthermore, new mechanistic insights responsible for maintaining factor V as an inactive procofactor will be discussed.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 15 October 2019
                Publication date (Electronic, collection): October 2019
                Volume: 11
                Issue: 10
                Electronic Location Identifier: e5918
                Affiliations
                [1 ] Research, Dorrington Medical Associates, Houston, USA
                [2 ] Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, USA
                [3 ] Medical Oncology and Hematology, Houston Methodist Hospital, Houston, USA
                [4 ] Critical Care, United General Hospital, Houston, USA
                Author notes
                Article
                DOI: 10.7759/cureus.5918
                PMC ID: 6857824
                SO-VID: 7e1e1cdb-9ad9-4ae3-99ee-b7071fbd6086
                Copyright © Copyright © 2019, Bello et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 8 October 2019
                Date accepted : 15 October 2019
                Categories
                Subject: Hematology
                Subject: Internal Medicine

                Keywords: factor v deficiency,prothrombin time,activated partial thromboplastin time,normal thrombin time,aortic valve replacement,fresh frozen plasma.
                Data availability:
                Keywords: factor v deficiency, prothrombin time, activated partial thromboplastin time, normal thrombin time, aortic valve replacement, fresh frozen plasma.

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