scholarly journals Unusual venous thrombosis associated with protein C deficiency

1988 ◽  
Vol 7 (3) ◽  
pp. 443-445 ◽  
Author(s):  
Myron E. Schwartz ◽  
Elizabeth B. Harrington ◽  
Jacob H. Rand
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Protein C Deficiency

The Frequency of Type I Heterozygous Protein S and Protein C Deficiency in 141 Unrelated Young Patients with Venous Thrombosis

1988 ◽  
Vol 59 (01) ◽  
pp. 018-022 ◽  
Author(s):  
C L Gladson ◽  
I Scharrer ◽  
V Hach ◽  
K H Beck ◽  
J H Griffin
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Antithrombin Iii ◽  
Young Patients ◽  
Protein S ◽  
Type I ◽  
Protein S Deficiency ◽  
Protein C Deficiency ◽  
Low Protein ◽  
S Deficiency

SummaryThe frequency of heterozygous protein C and protein S deficiency, detected by measuring total plasma antigen, in a group (n = 141) of young unrelated patients (<45 years old) with venous thrombotic disease was studied and compared to that of antithrombin III, fibrinogen, and plasminogen deficiencies. Among 91 patients not receiving oral anticoagulants, six had low protein S antigen levels and one had a low protein C antigen level. Among 50 patients receiving oral anticoagulant therapy, abnormally low ratios of protein S or C to other vitamin K-dependent factors were presented by one patient for protein S and five for protein C. Thus, heterozygous Type I protein S deficiency appeared in seven of 141 patients (5%) and heterozygous Type I protein C deficiency in six of 141 patients (4%). Eleven of thirteen deficient patients had recurrent venous thrombosis. In this group of 141 patients, 1% had an identifiable fibrinogen abnormality, 2% a plasminogen abnormality, and 3% an antithrombin III deficiency. Thus, among the known plasma protein deficiencies associated with venous thrombosis, protein S and protein C. deficiencies (9%) emerge as the leading identifiable associated abnormalities.

Concurrent Factor V Leiden and Protein C Deficiency Presenting as Mesenteric Venous Thrombosis

2016 ◽  
Vol 82 (4) ◽  
pp. 96-98 ◽  
Author(s):  
Lev N. Korovin ◽  
Mustafa Raoof ◽  
John B. Kettelle ◽  
James H. McClenathan ◽  
Jitesh A. Patel
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Factor V Leiden ◽  
Factor V ◽  
Mesenteric Venous Thrombosis ◽  
Protein C Deficiency

scholarly journals Protein C deficiency in a controlled series of unselected outpatients: an infrequent but clear risk factor for venous thrombosis (Leiden Thrombophilia Study) [see comments]

Blood ◽  
1995 ◽  
Vol 85 (10) ◽  
pp. 2756-2761 ◽  
Author(s):  
T Koster ◽  
FR Rosendaal ◽  
E Briet ◽  
FJ van der Meer ◽  
LP Colly ◽  
...  
Keyword(s):  
Relative Risk ◽  
Venous Thrombosis ◽  
Protein C ◽  
Protein S ◽  
Population Based ◽  
Repeated Measurements ◽  
Protein C Deficiency ◽  
Antithrombin Deficiency ◽  
Vein Thrombosis ◽  
Thrombosis Risk

A deficiency of protein C (PC), antithrombin, or protein S is strongly associated with deep-vein thrombosis in selected patients and their families. However, the strength of the association with venous thrombosis in the general population is unknown. This study was a population-based, patient-control study of 474 consecutive outpatients, aged less than 70 years, with a first, objectively diagnosed, episode of venous thrombosis and without an underlying malignant disease, and 474 healthy controls who matched for age and sex. Relative risks were estimated as matched odds ratios. Based on a single measurement, there were 22 (4.6%) patients with a PC deficiency (PC activity, less than 0.67 U/mL or PC antigen, less than 0.33 U/mL when using coumarins). Among the controls, the frequency was 1.5% (seven subjects). Thus, there is a threefold increase in risk of thrombosis in subjects with PC levels below 0.67 or 0.33 U/mL [matched odds ratio, 3.1; 95% confidence interval (CI), 1.4 to 7.0]. When a PC deficiency was based on two repeated measurements, the relative risk for thrombosis increased to 3.8 (95% CI, 1.3 to 10); when it was based on DNA-confirmation, the relative risk increased further to 6.5 (95% CI, 1.8 to 24). In addition, there was a gradient in thrombosis risk, according to PC levels. The results for antithrombin are similar to those for PC, although less pronounced (relative risk, 2.2; 95% CI, 1.0 to 4.7). We could not find an association between reduced total protein S (relative risk, 0.7; 95% CI, 0.3 to 1.8) or free protein S levels (relative risk, 1.6; 95% CI, 0.6 to 4.0) and thrombosis risk. Although not very frequent, PC and antithrombin deficiency are clearly associated with an increase in thrombosis risk.

Protein C deficiency in splanchnic venous thrombosis

1987 ◽  
Vol 82 (6) ◽  
pp. 1171-1174 ◽  
Author(s):  
David Green ◽  
Daniel R. Ganger ◽  
Andres T. Blei
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Protein C Deficiency

scholarly journals Apparent different thrombotic tendency in patients with factor V Leiden and protein C deficiency due to selection of patients

Blood ◽  
1996 ◽  
Vol 88 (11) ◽  
pp. 4205-4208 ◽  
Author(s):  
RP Lensen ◽  
FR Rosendaal ◽  
T Koster ◽  
CF Allaart ◽  
H de Ronde ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Age Of Onset ◽  
Factor V Leiden ◽  
Factor V ◽  
Protein C Deficiency ◽  
Apc Resistance ◽  
Selection Of Patients ◽  
Control Study ◽  
Selection Of

Both activated protein C (APC) resistance and protein C deficiency are associated with an increased risk for venous thrombosis. To assess their tendencies to venous thrombosis, we compared the median age of first venous thromboembolism in patients with factor V Leiden or protein C deficiency, who were identified either within unselected consecutive cases with a first deep venous thrombosis derived from a population-based case-control study, or identified by selection of patients with a deep venous thrombosis, who were referred for thrombophilIa work-up. The median age of onset for 92 unselected APC resistant cases was 43 years and for 13 unselected protein C-deficient cases 47 years. The median age at the first thrombotic event for 28 APC- resistant members of thrombophilia families was 29 years and for 50 protein C-deficient members of thrombophilia families 31.5 years. The median age of onset for all unselected patients (n=105) was 45 years of age (range, 16 to 69 years) and the median age of onset for all selected patients from the thrombophilia families (n=78) was 30.5 years (range, 16 to 67 years). These results show that within the case-control study and the family studies, the median age of onset is very similar in patients with APC resistance and patients with protein C deficiency. This suggests that APC resistance is not less severe with respect to risk of thrombosis than (heterozygous) protein C deficiency. In conclusion, the median age at which the first thrombosis occurs mainly depends on the way the patients are identified and not on the type of thrombophilia.

Protein-losing Enteropathy Caused by Mesenteric Venous Thrombosis With Protein C Deficiency

2000 ◽  
Vol 30 (1) ◽  
pp. 94-97 ◽  
Author(s):  
Isao Matsushita ◽  
Hiroyuki Hanai ◽  
Yoshihiko Sato ◽  
Hajime Arai ◽  
Takayuki Iida ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Mesenteric Venous Thrombosis ◽  
Protein C Deficiency ◽  
Protein Losing Enteropathy

Genotype–Phenotype Relationships in a Large French Cohort of Subjects with Inherited Protein C Deficiency

2020 ◽  
Vol 120 (09) ◽  
pp. 1270-1281
Author(s):  
Martine Alhenc-Gelas ◽  
Geneviève Plu-Bureau ◽  
Laetitia Mauge ◽  
Sophie Gandrille ◽  
Isabelle Présot ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Protein C ◽  
Anticoagulant Activity ◽  
Type I ◽  
Protein C Deficiency ◽  
Study Population ◽  
Heterozygous Carriers ◽  
Proc Gene ◽  
The Relationship ◽  
French Cohort

AbstractInherited protein C (PC) deficiency caused by mutations in the PROC gene is a well-known risk factor for venous thromboembolism. Few studies have investigated the relationship between PROC genotype and plasma or clinical phenotypes. We addressed this issue in a large retrospective cohort of 1,115 heterozygous carriers of 226 PROC pathogenic or likely pathogenic mutations. Mutations were classified in three categories according to their observed or presumed association with type I, type IIa, or type IIb PC deficiency. The study population comprised 876 carriers of type I category mutations, 55 carriers of type IIa category mutations, and 184 carriers of type IIb category mutations. PC anticoagulant activity significantly influenced risk of first venous thrombosis (p trend < 10−4). No influence of mutation category on risk of whole or unprovoked thrombotic events was observed. Both PC anticoagulant activity and genotype significantly influenced risk of venous thrombosis. Effect of detrimental mutations on plasma phenotype was ambiguous in several carriers, whatever the mutation category. Altogether, our findings confirm that diagnosing PC inherited deficiency based on plasma measurement may be difficult but show that diagnosis can be improved by PROC genotyping.

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