Discrepant ratios of arterial vs. venous thrombosis in hemophilias <scp>A</scp> and <scp>B</scp> as compared to <scp>FVII</scp> deficiency

Girolami, Antonio; Marinis, Giulia Berti de; Bertozzi, Irene; Peroni, Edoardo; Tasinato, Valentina; Lombardi, Anna Maria

doi:10.1111/ejh.12129

Cited by 11 publications

(13 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…2 –9 Factor VII levels were increased to at least 50 IU/dL in the current study, which was similar to previous trials. 22 –28 Thrombosis was reported in some cases after injection of rFVIIa in other studies but we did not encounter any thrombosis among our patients. 28 Also similar to other studies, study patients did not demonstrate any serious side effects and there was no significant difference between frequency of side effects.…”

Section: Discussionsupporting

confidence: 41%

A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients With Congenital Factor VII Deficiency

Faranoush

Abolghasemi

Toogeh

et al. 2014

Clin Appl Thromb Hemost

View full text Add to dashboard Cite

In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatment of congenital factor VII (FVII) deficiency, patients received either agent at 30 μg/kg, intravenously per week for 4 weeks, in a randomized fashion. The primary aim was to compare FVII:coagulation activity (FVII:C), 20 minutes after recombinant activated FVII (rFVIIa) injection, in the 2 groups. A secondary measure was self-reported bleeding. The median interquartile baseline range of the plasma level of activated FVII (FVIIa) activity in the 2 groups was 1.6 (1.1-14.0) IU/dL and 5.0 (1.1-25.5) IU/dL. All patients achieved levels of FVIIa (FVII:C) >30 IU/dL, 20 minutes after the injection of rFVIIa. Bleeding was similar between the 2 groups, with a comparable decrease in severity and frequency compared to the last month prior to treatment. AryoSeven is similar to NovoSeven in increasing postinjection FVIIa activity as well as in clinical safety and efficacy.

show abstract

Section: Discussionsupporting

confidence: 41%

A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients With Congenital Factor VII Deficiency

Faranoush

Abolghasemi

Toogeh

et al. 2014

Clin Appl Thromb Hemost

View full text Add to dashboard Cite

show abstract

“…Altogether, FVII deficiency does not ensure a sure protection from venous thromboembolisms. 34,35 The development of a FVII inhibitor in patients with FVII deficiency is a rare complication. It can be related to replacement therapy (about 1%-3% of treated patients, in most of the case in infants with a very severe phenotype) or linked to malignancy or autoimmune disease.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Factor VII Deficiency: From Basics to Clinical Laboratory Diagnosis and Patient Management

Sevenet

Kaczor

Depasse

2016

Clin Appl Thromb Hemost

View full text Add to dashboard Cite

Factor VII (FVII) deficiency is a rare inheritable bleeding disorder affecting 1/500 000 individuals. Clinical manifestations are heterogeneous, from asymptomatic to severe and potentially fatal bleeding. These clinical manifestations do not correlate well with FVII plasma levels. For this reason, FVII-deficient patient management during surgery or for long-term prophylaxis remains challenging. Laboratory testing for FVII activity is, however, the first-line method for FVII deficiency diagnosis and is helpful for managing patients in combination with clinical history. Additional testing consists of FVII immunoassay and genetic testing. Genetic abnormalities on the FVII gene are heterogeneous and can translate into quantitative or qualitative defects. Some of the latter can react differently with different thromboplastins; this can be misleading for the laboratory as no consensus exists at present on an FVII deficiency diagnosis methodology. Indeed, no single test is able to predict accurately the bleeding risk. This review provides a broad picture of inherited and acquired FVII deficiency with a particular focus on laboratory diagnosis.

show abstract

“…The only congenital coagulation disorder in which venous thrombosis is more frequent than arterial ones is FVII deficiency [28] The significance of these discrepancies and the observation that different clotting defects may yield both similar and different clinical patterns surely deserves further studies. The only congenital coagulation disorder in which venous thrombosis is more frequent than arterial ones is FVII deficiency [28] The significance of these discrepancies and the observation that different clotting defects may yield both similar and different clinical patterns surely deserves further studies.…”

Section: Discussionmentioning

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

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Discrepant ratios of arterial vs. venous thrombosis in hemophilias A and B as compared to FVII deficiency

Abstract: Hemophilia A and hemophilia B do not protect from arterial occlusions (mainly acute coronary syndromes), whereas they assure some protection from venous thrombosis. The opposite seems true for FVII deficiency. The potential significance of this discrepancy is discussed.

Cited by 11 publications

References 36 publications

A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients With Congenital Factor VII Deficiency

A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients With Congenital Factor VII Deficiency

Factor VII Deficiency: From Basics to Clinical Laboratory Diagnosis and Patient Management

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

Contact Info

Product

Resources

About