Discrepant ratios of arterial versus venous thrombosis in hemophilia A as compared with hemophilia B

Girolami, Antonio; Bertozzi, Irene; Marinis, Giulia Berti de; Tasinato, Valentina; Sambado, Luisa

doi:10.1007/s11239-013-0943-7

Cited by 12 publications

(16 citation statements)

References 32 publications

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“…It indicateds only that the protection is not absolute. The same has been demonstrated for the hemophilias and other congenital clotting factor deficiencies, notably F VII deficiency [24][25][26][27][28] .…”

Section: Discussionsupporting

confidence: 54%

Associated risk factors and arterial occlusions in patients with von Willebrand disease: Analysis of the literature and report of two cases

Girolami

Ferrari

Bertomoro

et al. 2014

CRIM

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Objective: To investigate the features of all reported cases of arterial occlusion which occurred in patients with Von Willebrand Disease. Patients and methods: Personal records were re-evaluated together with a time-unlimited Pubmed search using pertinent keywords. Occlusive events had to be documented by objective means. All cases of myocardial infarction and other acute coronary syndromes were considered. Cases of angina were instead excluded. Results: 56 patients with arterial occlusion were gathered from the literature (44 cases of acute coronary syndromes and 12 cases of ischemic stroke. Two additional cases were obtained from personal records (1 case of myocardial infarction and 1 case of unstable angina) for a total of 58 patients. Peripheral arterial thrombosis has never been described in von Willebrand disease patients. The majority of these patients presented common risk factors such as smoking, hypertension, diabeties, dyslipidemia, obesity. Replacement therapy, contrary to what seen in hemophilias, had instead a minor role if any. Conclusions: von Willebrand disease is unable to protect from atherosclerosis and atherothrombosis. The common risk factors for arterial disease such as hypertension, diabetiesdyslipidemia and smoking are able to neutralize the potential protective effect of VW Factor deficiency. Despite this, the prevalence of arterial occlusions in von Willebrand disease appears decreased in comparison with the general populations at least in some countries.

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Section: Discussionsupporting

confidence: 54%

Associated risk factors and arterial occlusions in patients with von Willebrand disease: Analysis of the literature and report of two cases

Girolami

Ferrari

Bertomoro

et al. 2014

CRIM

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“…Finally, although a relatively rare event, patients with haemophilia (either congenital or acquired) are not invulnerable to the development of thrombosis and associated anticoagulant treatment, particularly during medical interventions . Although DOACs are not routinely used in such settings, or even in haemophilia patients per se, they may be so used in the future.…”

Section: Introductionmentioning

confidence: 99%

Reducing the effect of DOAC interference in laboratory testing for factor VIII and factor IX: A comparative study using DOAC Stop and andexanet alfa to neutralize rivaroxaban effects

et al. 2020

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Introduction Investigation of factors (F) VIII and IX is common, with testing important for diagnosis or exclusion of haemophilia A or B, associated acquired conditions and factor inhibitors. Rivaroxaban, a common direct anti‐Xa agent, causes significant interference in clotting assays, including substantial false reduction of factor levels. Aim To assess whether rivaroxaban‐induced interference of FVIII and FIX testing could be neutralized. Materials and methods An international, cross‐laboratory exercise for FVIII (n = 84) and FIX (n = 74), using four samples: (A) pool of normal plasma; (B) pool spiked with rivaroxaban (200 ng/mL); (C) rivaroxaban sample subsequently treated with ‘DOAC Stop’ and; (D) rivaroxaban sample treated with andexanet alfa (200 μg/mL). Testing performed blind to sample type. Results All laboratories reported normal FIX and 94% reported normal FVIII in the pool sample. Instead, 55% and 95%, respectively, reported abnormal FIX and FVIII levels for the rivaroxaban sample. DOAC Stop treatment evidenced a correction in most laboratories (100% reported normal FIX and 86% normal FVIII). Andexanet alfa provided intermediate results, with many laboratories still reporting abnormal results (59% for FVIII, 18% for FIX). We also identified reagent‐specific issues. Conclusions As expected, rivaroxaban caused false low values of FVIII and FIX. This might lead to increased testing to identify the cause of low factor levels and potentially lead to false identification of (mild) haemophilia A or B if unrecognized by clinicians/laboratories. DOAC Stop effectively neutralized the rivaroxaban effect, but andexanet alfa less so, with reagent‐related effects evident, and thus, false low values sometimes persisted.

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“…Noncatheter-associated venous thrombosis seems rare in VWD [5,22]. That this is so is well demonstrated by the observation that no case is listed in the 1983 review by Goodnough et al [23] on this subject.…”

Section: Discussionmentioning

confidence: 96%

“…This is surely true for the hemophilias and apparently it is also true for von Willebrand disease (vWD) [2][3][4][5] (A. Girolami, S. Ferrari, A. Bertomoro, E. Peroni, L. Sambado, A. Casonato, in preparation). This is surely true for the hemophilias and apparently it is also true for von Willebrand disease (vWD) [2][3][4][5] (A. Girolami, S. Ferrari, A. Bertomoro, E. Peroni, L. Sambado, A. Casonato, in preparation).…”

Section: Introductionmentioning

confidence: 99%

Venous thrombosis in von Willebrand disease as observed in one centre and as reported in the literature

Girolami

Tasinato²,

Sambado³

et al. 2015

Blood Coagulation &Amp; Fibrinolysis

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The aim of this article was to investigate the prevalence of venous thrombosis in patients with von Willebrand disease. Personal records on 486 patients were reevaluated together with a time unlimited PubMed search. The venous thrombotic event had to be proven by objective means. Only cases of congenital von Willebrand disease were taken into consideration and all types of the diseases were included. No case of venous thrombosis was reported in our cohort of patients. On the contrary, 33 patients with proven venous thrombosis were gathered from the literature (17 cases of deep venous thrombosis with or without pulmonary embolism; isolated pulmonary embolism was seen in seven instances, superficial veins or portal system thrombosis was present in the remaining cases). Associated risk factors, mainly replacement therapy, were present in 26 cases. Therapeutic approach was usually based on heparin and Coumadin. Overall results were fair or good, as no fatalities occurred.

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Discrepant ratios of arterial versus venous thrombosis in hemophilia A as compared with hemophilia B

Cited by 12 publications

References 32 publications

Associated risk factors and arterial occlusions in patients with von Willebrand disease: Analysis of the literature and report of two cases

Associated risk factors and arterial occlusions in patients with von Willebrand disease: Analysis of the literature and report of two cases

Reducing the effect of DOAC interference in laboratory testing for factor VIII and factor IX: A comparative study using DOAC Stop and andexanet alfa to neutralize rivaroxaban effects

Venous thrombosis in von Willebrand disease as observed in one centre and as reported in the literature

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