Homozygous FVII deficiencies with different reactivity towards tissue thromboplastins of different origin

Girolami, Antonio; Scarparo, Pamela; Bonamigo, Emanuela; Treleani, Martina; Lombardi, Annalina

doi:10.1179/1024533212z.000000000144

Cited by 11 publications

(12 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In this variant, FVII activity was 6-8% of normal using rabbit brain thromboplastins (r-thromboplastin) and normal using ox brain throm-boplastins. Intermediate values around 30-40% of normal were obtained using human placenta or human recombinant thromboplastin (h-thromboplastin) was used [158]. Those findings have been confirmed by other studies [159,160,161].…”

Section: Measurement Of Fvii By Using Different Thromboplastinssupporting

confidence: 76%

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Chaireti¹

2013

View full text Add to dashboard Cite

Section: Measurement Of Fvii By Using Different Thromboplastinssupporting

confidence: 76%

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Chaireti¹

2013

View full text Add to dashboard Cite

“…Prothrombin time reagents can contain heparin neutralisers and vary in the source of tissue factor (TF) and phospholipids used. The source and composition of TF can influence measured levels in some cases 43,44 and whether the reagent is sensitive to direct factor Xa inhibitors (DFXaI) 45 or LA 46 …”

Section: Investigation Of a Bleeding Tendencymentioning

confidence: 99%

Guidelines on the laboratory aspects of assays used in haemostasis and thrombosis

et al. 2020

View full text Add to dashboard Cite

“…As seen in FX deficiency, variants were discovered that showed different reactivity toward tissue thromboplastins and other activating agents of different origin. 35,36 The most important among these variants is FVII Padua (Arg304Gln), which shows low FVII activity levels using rabbit brain thromboplastin but normal levels using ox brain thromboplastin. Thromboplastin of human origin (brain, placenta) yields intermediate values (approximately 30-35% of normal).…”

Section: Study Of Variants and Classificationmentioning

confidence: 99%

The Story of Serum Prothrombin Conversion Accelerator, Proconvertin, Stable Factor, Cothromboplastin, Prothrombin Accelerator or Autoprothrombin I, and Their Subsequent Merging into Factor VII

Girolami

Cosi

Sommer

et al. 2015

Semin Thromb Hemost

View full text Add to dashboard Cite

Factor VII (FVII) deficiency is one of the two congenital coagulation disorders that was not discovered by the description of a new bleeding patient whose clotting pattern did not fit the blood coagulation knowledge of the time (the other is factor XIII deficiency). The existence of an additional factor capable of accelerating the conversion of prothrombin into thrombin was suspected before 1951, the year in which the first family with FVII deficiency was discovered. As several investigators were involved in the discovery of FVII deficiency from both sides of the Atlantic, several different names were tentatively suggested to define this entity, namely stable factor (in contrast with labile factor or FV), cothromboplastin, proconvertin, serum prothrombin conversion accelerator, prothrombin acceleration, and autoprothrombin I. The last term was proposed by those who denied the existence of this new entity, which was instead considered to be a derivate of prothrombin activation, namely autoprothrombin. The description of several families, from all over the world, of the same defect, however clearly demonstrated the singularity of the condition. Factor VII was then proposed to define this protein. In subsequent years, several variants were described with peculiar reactivity toward tissue thromboplastins of different origin. Molecular biology techniques demonstrated several gene mutations, usually missense mutations, often involving exon 8 of the FVII gene. Later studies dealt with the relation of FVII with tissue factor and activated FVII (FVIIa). The evaluation of circulating FVIIa was made possible by the use of a truncated form of tissue factor, which is only sensitive to FVIIa present in the circulation. The development of FVII concentrates, both plasma derived and recombinant, has facilitated therapeutic management of FVII-deficient patients. The use of FVIIa concentrates was noted to be associated with the occasional occurrence of thrombotic events, mainly venous. Total or partial liver transplants have been performed with success in these patients and have "cured" their deficiencies. Prenatal diagnosis has also been performed and recent research involves the development of inhibitors of FVII + tissue factor complex or of FVIIa. This approach, if successful, could provide another antithrombotic therapeutics tool. The story of FVII well summarizes the efforts of both theoretical and clinical approaches in the characterization of a coagulation disorder, that is, among the rare bleeding conditions, most frequently encountered in clinical practice.

show abstract

Homozygous FVII deficiencies with different reactivity towards tissue thromboplastins of different origin

Cited by 11 publications

References 22 publications

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Guidelines on the laboratory aspects of assays used in haemostasis and thrombosis

The Story of Serum Prothrombin Conversion Accelerator, Proconvertin, Stable Factor, Cothromboplastin, Prothrombin Accelerator or Autoprothrombin I, and Their Subsequent Merging into Factor VII

Contact Info

Product

Resources

About