Risk Factors Associated with Postpartum Ovarian Vein Thrombosis

SummaryThrombosis of the ovarian vein is a remarkable process occuring within a few days of labor in 1:500-1:2000 women. Its presentation is characterized by fever, abdominal pain and occasionally by a palpable abdominal mass that in earlier years sometimes lead to explorative laparotomy. With the advent of modern imaging techniques the diagnosis can be made relatively easily. The pathogenesis has been attributed to an infectious process expanding from the uterus to the right ovarian vein and stasis. A predisposition towards thrombosis has not been so far explored. In this study we retrospectively analysed the clinical features, diagnosis and treatment of 22 patients with objective documentation of post partum ovarian vein thrombosis (POVT) and assessed potential risk factors. In 11 of the 22 patients (50%) inherited prothrombotic risk factors were detected as follows: 4 were heterozygous for factor V G1691 A, 2 had protein S deficiency, one had protein S deficiency and was heterozygous for factor V G1691A, and 4 were homozygous for MTHFR C677T. Eight of the 11 patients who bore a prothrombotic predisposition underwent cesarean section. Taken together, the data suggest that POVT may result from the combined effect of an infection, cesarean section and a prothrombotic tendency.

Download Full-text

Different Risks of Thrombosis in Four Coagulation Defects Associated With Inherited Thrombophilia: A Study of 150 Families

Blood ◽

10.1182/blood.v92.7.2353 ◽

1998 ◽

Vol 92 (7) ◽

pp. 2353-2358 ◽

Cited By ~ 245

Author(s):

Ida Martinelli ◽

Pier Mannuccio Mannucci ◽

Valerio De Stefano ◽

Emanuela Taioli ◽

Valentina Rossi ◽

...

Keyword(s):

Protein C ◽

Factor V Leiden ◽

Protein S ◽

Factor V ◽

Superficial Vein ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Superficial Vein Thrombosis ◽

Coagulation Defects

AbstractDeficiency of the naturally occurring anticoagulant proteins, such as antithrombin, protein C and protein S, and activated protein C resistance due to the factor V Leiden gene mutation is associated with inherited thrombophilia. So far, no direct comparison of the thrombotic risk associated with these genetic defects is available. In this study, we wish to compare the lifetime probability of developing thrombosis, the type of thrombotic symptoms, and the role of circumstantial triggering factors in 723 first- and second-degree relatives of 150 index patients with different thrombophilic defects. We found higher risks for thrombosis for subjects with antithrombin (risk ratio 8.1, 95% confidence interval [CI], 3.4 to 19.6), protein C (7.3, 95% CI, 2.9 to 18.4) or protein S deficiency (8.5, 95% CI, 3.5 to 20.8), and factor V Leiden (2.2, 95% CI, 1.1 to 4.7) than for individuals with normal coagulation. The risk of thrombosis for subjects with factor V Leiden was lower than that for those with all three other coagulation defects (0.3, 95% CI, 0.1 to 1.6), even when arterial and superficial vein thromboses were excluded and the analysis was restricted to deep vein thrombosis (0.3, 95% CI, 0.2 to 0.5). No association between coagulation defects and arterial thrombosis was found. The most frequent venous thrombotic manifestation was deep vein thrombosis with or without pulmonary embolism (90% in antithrombin, 88% in protein C, 100% in protein S deficiency, and 57% in factor V Leiden), but a relatively mild manifestation such as superficial vein thrombosis was common in factor V Leiden (43%). There was a predisposing factor at the time of venous thromboembolism in approximately 50% of cases for each of the four defects. In conclusion, factor V Leiden is associated with a relatively small risk of thrombosis, lower than that for antithrombin, protein C, or protein S deficiency. In addition, individuals with factor V Leiden develop less severe thrombotic manifestations, such as superficial vein thrombosis.

Download Full-text

Heerlen polymorphism associated with type III protein S deficiency and factor V Leiden mutation in a Polish patient with deep vein thrombosis

Blood Coagulation & Fibrinolysis ◽

10.1097/mbc.0b013e328365032c ◽

2014 ◽

Vol 25 (1) ◽

pp. 84-85 ◽

Cited By ~ 4

Author(s):

Ewa Wypasek ◽

Daniel P. Potaczek ◽

Martine Alhenc-Gelas ◽

Anetta Undas

Keyword(s):

Deep Vein Thrombosis ◽

Factor V Leiden ◽

Protein S ◽

Factor V ◽

Protein S Deficiency ◽

Factor V Leiden Mutation ◽

Vein Thrombosis ◽

S Deficiency ◽

Deep Vein ◽

Polish Patient

Download Full-text

Deep Vein Thrombosis in Protein S Deficiency

Journal of Nepal Medical Association ◽

10.31729/jnma.137 ◽

2010 ◽

Vol 49 (177) ◽

Author(s):

A Joshi ◽

J P Jaiswal

Keyword(s):

Deep Vein Thrombosis ◽

Vitamin K ◽

Rare Condition ◽

Protein S ◽

Factor V ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

Hepatic Diseases ◽

S Deficiency ◽

Deep Vein

Protein S is a vitamin K-dependent anticoagulant protein. It functions as a cofactor of activated protein C to inactivate activated factor V (FVa) and activated factor VIII (FVIIIa). Its deficiency is a rare condition and can lead to deep vein thrombosis, pulmonary embolism or stroke. It is often treated with long-term anti-coagulant therapy. Protein S deficiency may be hereditary or acquired; the latter is usually due to hepatic diseases or a vitamin K deficiency. Protein S deficiency manifests as an autosomal dominant trait; manifestations of thrombosis are observed in both heterozygous and homozygous genetic deficiencies of protein S. This case report is of DVT due to Protein S deficiency in a 53 year old male. Venous Doppler was used to diagnose DVT and free Protein S level measured by ELISA. IVC filter was placed on the third day of admission. Keywords: antithrombotic, deep venous thrombosis, inferior venacaval filter, protein S.

Download Full-text

Different Risks of Thrombosis in Four Coagulation Defects Associated With Inherited Thrombophilia: A Study of 150 Families

Blood ◽

10.1182/blood.v92.7.2353.2353_2353_2358 ◽

1998 ◽

Vol 92 (7) ◽

pp. 2353-2358 ◽

Cited By ~ 4

Author(s):

Ida Martinelli ◽

Pier Mannuccio Mannucci ◽

Valerio De Stefano ◽

Emanuela Taioli ◽

Valentina Rossi ◽

...

Keyword(s):

Protein C ◽

Factor V Leiden ◽

Protein S ◽

Factor V ◽

Superficial Vein ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Superficial Vein Thrombosis ◽

Coagulation Defects

Deficiency of the naturally occurring anticoagulant proteins, such as antithrombin, protein C and protein S, and activated protein C resistance due to the factor V Leiden gene mutation is associated with inherited thrombophilia. So far, no direct comparison of the thrombotic risk associated with these genetic defects is available. In this study, we wish to compare the lifetime probability of developing thrombosis, the type of thrombotic symptoms, and the role of circumstantial triggering factors in 723 first- and second-degree relatives of 150 index patients with different thrombophilic defects. We found higher risks for thrombosis for subjects with antithrombin (risk ratio 8.1, 95% confidence interval [CI], 3.4 to 19.6), protein C (7.3, 95% CI, 2.9 to 18.4) or protein S deficiency (8.5, 95% CI, 3.5 to 20.8), and factor V Leiden (2.2, 95% CI, 1.1 to 4.7) than for individuals with normal coagulation. The risk of thrombosis for subjects with factor V Leiden was lower than that for those with all three other coagulation defects (0.3, 95% CI, 0.1 to 1.6), even when arterial and superficial vein thromboses were excluded and the analysis was restricted to deep vein thrombosis (0.3, 95% CI, 0.2 to 0.5). No association between coagulation defects and arterial thrombosis was found. The most frequent venous thrombotic manifestation was deep vein thrombosis with or without pulmonary embolism (90% in antithrombin, 88% in protein C, 100% in protein S deficiency, and 57% in factor V Leiden), but a relatively mild manifestation such as superficial vein thrombosis was common in factor V Leiden (43%). There was a predisposing factor at the time of venous thromboembolism in approximately 50% of cases for each of the four defects. In conclusion, factor V Leiden is associated with a relatively small risk of thrombosis, lower than that for antithrombin, protein C, or protein S deficiency. In addition, individuals with factor V Leiden develop less severe thrombotic manifestations, such as superficial vein thrombosis.

Download Full-text

Homozygosity for the Protein S Heerlen Allele Is Associated with Type I PS Deficiency in a Thrombophilic Pedigree with Multiple Risk Factors

Thrombosis and Haemostasis ◽

10.1055/s-0037-1613764 ◽

2000 ◽

Vol 83 (01) ◽

pp. 102-106 ◽

Cited By ~ 17

Author(s):

Yolanda Espinosa-Parrilla ◽

Gemma Navarro ◽

Marta Morell ◽

Eugènia Abella ◽

Xavier Estivill ◽

...

Keyword(s):

Risk Factors ◽

Protein S ◽

Type I ◽

Factor V ◽

Type Iii ◽

Vein Thrombosis ◽

Multiple Risk Factors ◽

S Deficiency ◽

Recurrent Deep Vein Thrombosis ◽

Deep Vein

SummaryThe multifactorial character of thrombotic disease is shown in a Spanish pedigree in which the propositus, with recurrent deep vein thrombosis, inherited the factor V R/Q506 mutation, the prothrombin 20210G/A variant and type III Protein S deficiency. Among 14 relatives carrying one or two of these three risk factors, thrombosis is present in a heterozygote for R/Q506 and in another for 20210G/A, who also had slightly positive antiphospholipid antibodies. Type I PS deficiency was also found in a young asymptomatic woman. PROS1 analysis showed coexistence of type III and type I PS deficiency to be associated with heterozygosity and homozygosity, respectively, for the P460 or PS Heerlen allele of the S/P460 variant. Analysis of PS values in this and other pedigrees segregating this variant revealed that not only free but also mean total PS levels are slightly but significantly lower in the SP460 heterozygotes than in the SS460 homozygotes. These findings strongly suggest a role of the P460 variant in the expression of the PS deficient phenotype.

Download Full-text

Utility of Thrombin-Generation Assay in the Screening of Factor V G1691A (Leiden) and Prothrombin G20210A Mutations and Protein S Deficiency

Clinical Chemistry ◽

10.1373/clinchem.2005.063339 ◽

2006 ◽

Vol 52 (4) ◽

pp. 665-670 ◽

Cited By ~ 22

Author(s):

Nathalie Hézard ◽

Lobna Bouaziz-Borgi ◽

Marie-Geneviève Remy ◽

Philippe Nguyen

Keyword(s):

Risk Factors ◽

Thrombin Generation ◽

Factor V Leiden ◽

Protein S ◽

Prothrombin G20210a ◽

Factor V ◽

Protein S Deficiency ◽

Roc Curve Analysis ◽

Thrombin Generation Assay ◽

S Deficiency

Abstract Background: The thrombin-generation assay has a variety of clinical uses, including diagnosis of thromboembolism-related disease, and particular profiles are associated with thrombophilic risk factors. The aim of this study was to evaluate the use of this assay in screening and identifying patients who require specific thrombophilic testing. Methods: We used a 2-step approach to perform specific thrombophilic testing and thrombin-generation assays on 169 consecutive patients. The first step was to identify particular profiles of thrombin generation corresponding to each type of thrombophilic risk factor and to determine the pertinent variables related to thrombin generation. We then performed ROC curve analysis for each predefined variable to determine the relevant cutoffs for identification of patients in need of further testing (negative predictive value, 100%). Results: Suggestive profiles were seen in factor V Leiden (n = 49) and prothrombin (n = 12) mutations and in protein S deficiency (n = 12). ROC curves showed that factor V Leiden may be excluded when the difference between lag times obtained in the absence and presence of activated protein C (APC) is >1.5 min and that prothrombin G20210A may also be excluded when the peak thrombin concentration is ≤426 nmol/L. In addition, protein S deficiency may be excluded when the percentage of APC-induced endogenous thrombin potential inhibition is >63%. Conclusion: The thrombin-generation assay represents a promising tool for screening thrombophilic risk factors, particularly in patients who are carriers of factor V Leiden or prothrombin G20210A mutations and patients with protein S deficiency.

Download Full-text

Post-Partum Ovarian Vein Thrombosis: Combined Effect of Infection and Factor V Leiden Mutation

Turkish Journal of Hematology ◽

10.4274/tjh.2014.0266 ◽

2015 ◽

Vol 32 (1) ◽

pp. 80-81

Author(s):

H. El Farran ◽

A. G. Haddad ◽

A. H. Radwan ◽

A. H. Nassar ◽

R. Hourani ◽

...

Keyword(s):

Post Partum ◽

Factor V Leiden ◽

Combined Effect ◽

Factor V ◽

Ovarian Vein ◽

Factor V Leiden Mutation ◽

Vein Thrombosis ◽

Ovarian Vein Thrombosis

Download Full-text

Elevated Levels of Prothrombin Activation Fragment 1+2 in Plasma from Patients with Heterozygous Arg506 to Gin Mutation in the Factor V Gene (APC-Resistance) and/or Inherited Protein S Deficiency

Thrombosis and Haemostasis ◽

10.1055/s-0038-1650259 ◽

1996 ◽

Vol 75 (02) ◽

pp. 270-274 ◽

Cited By ~ 57

Author(s):

Benget Zöller ◽

Johan Holm ◽

Peter Svensson ◽

Björn Dahlbäck

Keyword(s):

Plasma Concentration ◽

Protein C ◽

Protein S ◽

Factor V ◽

Protein S Deficiency ◽

Apc Resistance ◽

S Deficiency ◽

Increased Risk ◽

Factor V Gene ◽

V Gene

SummaryInherited resistance to activated protein C (APC-resistance), caused by a point mutation in the factor V gene leading to replacement of Arg(R)506 with a Gin (Q), and inherited protein S deficiency are associated with functional impairment of the protein C anticoagulant system, yielding lifelong hypercoagulability and increased risk of thrombosis. APC-resistance is often an additional genetic risk factor in thrombosis-prone protein S deficient families. The plasma concentration of prothrombin fragment 1+2 (F1+2), which is a marker of hyper-coagulable states, was measured in 205 members of 34 thrombosis-prone families harbouring the Arg506 to Gin mutation (APC-resistance) and/or inherited protein S deficiency. The plasma concentration of F1+2 was significantly higher both in 38 individuals carrying the FV:Q506 mutation in heterozygous state (1.7 ± 0.7 nM; mean ± SD) and in 48 protein S deficient cases (1.9 ± 0.9 nM), than in 100 unaffected relatives (1.3 ±0.5 nM). Warfarin therapy decreased the F1+2 levels, even in those four patients who had combined defects (0.5 ± 0.3 nM). Our results agree with the hypothesis that individuals with APC-resistance or protein S deficiency have an imbalance between pro- and anti-coagulant forces leading to increased thrombin generation and a hypercoagulable state.

Download Full-text