Treatment tailoring for factor V deficient patients and perioperative management using global hemostatic coagulation assays

Levy‐Mendelovich, Sarina; Barg, Assaf Arie; Rosenberg, Nurit; Avishai, Einat; Luboshitz, Jacob; Misgav, Mudi; Kenet, Gili; Livnat, Tami

doi:10.1016/j.bcmd.2018.01.002

Cited by 6 publications

(8 citation statements)

References 17 publications

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“…Factor V deficiency is usually diagnosed by observing an elevated PT, PTT and INR followed by a low factor V assay. Other diagnostic modalities that can help guide transfusion of blood products in the OR are TG and ROTEM . In this patient, FFP was successfully used before, during and after the surgery to correct his coagulopathy.…”

Section: Results and Conclusionmentioning

confidence: 96%

On‐pump CABG in a patient with severe factor V deficiency

et al. 2019

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Section: Results and Conclusionmentioning

confidence: 96%

On‐pump CABG in a patient with severe factor V deficiency

et al. 2019

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“…Advances in the management and treatment of haemophilia include the development of new therapeutic products, interventions and monitoring techniques. [1][2][3][4][5][6] The introduction of these and other developments into clinical practice will necessarily generate complexities, in addition to existing unmet needs requiring attention by physicians who treat patients with haemophilia. To gain an understanding of these needs, it is necessary to implement a process that surveys a wide range of expert frontline clinicians who have knowledge of current drivers in haemophilia care.…”

Section: Haemophilia Clinical Care and Research Needs: Assessing Priomentioning

confidence: 99%

“…The literature contains only a few reports of heavy menstrual bleeding in patients with severe FV deficiency. [3][4][5][6]9 Treatment regimens with FFP, antifibrinolytics, desmopressin and oral contraceptives…”

mentioning

confidence: 99%

“…have not yet been optimized, and it can be difficult to control heavy menstrual bleeding episodes with these methods. [3][4][5][6] Administration of additional hormonal manipulation may be useful in managing bleeding but has not been proven to be optimal treatment for heavy menstrual bleeding. 10 Bypassing-agent monotherapy has been used with variable success in most patients with severe FV deficiency who have inhibitors or are allergic to FFP.…”

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confidence: 99%

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Recombinant FVIIa therapy for heavy menstrual bleeding in patients with severe FV deficiency

Kaya

2018

Haemophilia

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“…Our group and others have previously demonstrated that thrombin generation is a sensitive assay for monitoring the use of rFVIIa and can be used as a tool for predicting rFVIIa concentrations for surgical interventions in haemophilic as well as non-haemophilic bleeding patients. [20][21][22] Since GT patients have impaired thrombin generation capacity, 23,24 we used thrombin generation assay to evaluate their response to rFVIIa.…”

Section: Introductionmentioning

confidence: 99%

Low Concentrations of Recombinant Factor VIIa May Improve the Impaired Thrombin Generation of Glanzmann Thrombasthenia Patients

et al. 2018

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Introduction Glanzmann thrombasthenia (GT) is a rare bleeding disorder. The disease is caused by the lack or dysfunction of platelet membrane glycoprotein IIb/IIIa (integrin αIIbβ3) which is essential for platelet aggregation. Bleeding episodes are usually managed by platelet transfusions. Recombinant activated factor VII (rFVIIa) is a common adjunct or alternative treatment option. Objective This article evaluates GT patients' response to increasing concentrations of rFVIIa using an ex vivo thrombin generation assay to elaborate the knowledge in which rFVIIa treats a platelet dysfunction for bleeding episodes and preoperative management. Materials and Methods Twenty-four GT patients in a non-bleeding state were enrolled into the study. Thrombin generation was measured in platelet-rich plasma (PRP) in the presence of 0.7 to 7.0 µg/mL rFVIIa. Clinical data of rFVIIa used to treat GT patients' bleeding episodes was collected, and patients' follow-up course was documented. Results Thrombin generation was significantly decreased in GT patients compared with controls. An individual response to rFVIIa spiking was noted in GT patients' PRP. In the majority of patients, a significant improvement in thrombin generation was already demonstrated with low concentrations (0.7 µg/mL) of rFVIIa. Conclusion Thrombin generation is improved in the majority of GT patients following ex vivo spiking with rFVIIa. The magnitude of this improvement is individual and was noted at low concentrations of rFVIIa. There is a need for a prospective clinical trial to find the optimal doses or rFVIIa for treatment of GT patients.

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Treatment tailoring for factor V deficient patients and perioperative management using global hemostatic coagulation assays

Cited by 6 publications

References 17 publications

On‐pump CABG in a patient with severe factor V deficiency

On‐pump CABG in a patient with severe factor V deficiency

Recombinant FVIIa therapy for heavy menstrual bleeding in patients with severe FV deficiency

Low Concentrations of Recombinant Factor VIIa May Improve the Impaired Thrombin Generation of Glanzmann Thrombasthenia Patients

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