Effectiveness of longterm oral anticoagulant treatment in preventing thrombosis in hereditary protein S deficiency

SummaryProtein S, a vitamin K-dependent coagulation factor, is involved in the regulation of the anticoagulant activity of activated protein C.Using an immunoradiometric assay for total protein S in plasma we identified 14 patients (7 male and 7 female) in three unrelated Dutch families as fulfilling the criteria for an isolated protein S deficiency. In 9 patients who were not receiving oral anticoagulant treatment the mean total protein S antigen concentration was 0.50 ± 0.08 U/ml (± S.D.) and the calculated free protein S concentration was 0.15 ± 0.01 U/ml (± S.D.). In the five patients who were on oral anticoagulant treatment the mean total protein S antigen was 0.23 ± 0.05 U/ml (± S.D.).Seven of the 14 patients had a history of venous thromboembolism occurring at a mean age of 25 years and often without an apparent cause. Protein S deficiency is inherited as an autosomal dominant trait.

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Recurrent venous thrombosis during direct oral anticoagulant therapy in a patient with protein S deficiency

The Journal of Medical Investigation ◽

10.2152/jmi.66.182 ◽

2019 ◽

Vol 66 (1.2) ◽

pp. 182-184 ◽

Cited By ~ 3

Author(s):

Shusuke Yagi ◽

Kumiko Kagawa ◽

Eiki Fujimoto ◽

Masataka Sata

Keyword(s):

Venous Thrombosis ◽

Anticoagulant Therapy ◽

Oral Anticoagulant ◽

Oral Anticoagulant Therapy ◽

Protein S ◽

Direct Oral Anticoagulant ◽

Protein S Deficiency ◽

S Deficiency ◽

Recurrent Venous Thrombosis

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ANTICOAGULANT AND ANTIGENIC LEVELS OF PROTEIN C AND PROTEIN S IN PATIENTS ON STABILIZED ORAL ANTICOAGULANT TREATMENT

10.1055/s-0038-1644286 ◽

1987 ◽

Author(s):

A D'Angelo ◽

F Gilardoni ◽

M P Seveso ◽

P Poli ◽

R Quintavalle ◽

...

Keyword(s):

Vitamin K ◽

Oral Anticoagulant ◽

Protein C ◽

Protein S ◽

Reference Interval ◽

Factor Ix ◽

Activity Levels ◽

Anticoagulant Treatment ◽

Oral Anticoagulant Treatment ◽

Congenital Deficiency

Isolated deficiencies of protein C and protein S, two vitamin K-dependent plasma proteins, constitute about 70% of the congenital abnormalities of blood coagulation observed in patients with recurrent venous thrombosis beLow the age of 40. The laboratory diagnosis of congenital deficiency of these proteins represents a major problem since a large proportion of patients are on oral anticoagulation (OA) at the time the deficiencies are suspected.Under these circumstances the availability of a reference interval obtained in patients on stabilized OA has proven useful.Functional (C) and antigenic levels (Ag) of protein C, protein S, factor IX and II were estimated in 136 patients on stabilized OA, subdivided according to the degree of anticoagulation (Internatio nal Normalized Ratio, INR).The results indicate that with increasing anticoagulation the activity levels of all the vitamin K-dependent factors decrease to a greater extent than the corresponding antigenic levels. At variance with the other factors, total protein S antigen levels are only moderately reduced by OA with protein S anticoagulant activi ty comparing well to factor IX clotting activity. These data suggest the possibility of identifying both quantitative and qualita tive deficiencies of protein C and protein S in patients on oral anticoagulant treatment.

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A Rare Case of Portal Vein Thrombosis due to Protein S deficiency in a patient with Decompensated Cryptogenic CLD with Portal Hypertension, Completely Recanalized by Single Oral Anticoagulant-Rivaroxaban

10.36811/ijho.2020.110007 ◽

2020 ◽

pp. 29-37

Author(s):

Richmond Ronald Gomes

Keyword(s):

Portal Hypertension ◽

Liver Disease ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Oral Anticoagulant ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Hepatic Portal

Venous thromboembolic diseases are a group of heterogeneous diseases with different clinical forms and prognosis. Abdominal venous thrombosis may present either as Budd-Chiari syndrome (BCS) caused by hepatic vein or proximal inferior vena cava (IVC) obstruction or as an extra hepatic portal obstruction (EHPVO) caused by Portal vein thrombosis or mesenteric vein thrombosis. Portal vein thrombosis (PVT) is a rare form of venous thrombosis that affects the hepatic portal vein flow, which can lead to portal hypertension. Treatment of PVT includes anticoagulants, thrombolysis, and insertion of shunts, bypass surgery, and liver transplantation. Single anticoagulation therapy can be associated with a reduction in new thrombotic episodes. Here we experienced a 23 year old young lady with history of recent intrauterine death (IUD) diagnosed as PVT provoked by protein S deficiency with newly diagnosed decompensated cryptogenic chronic liver disease with portal hypertension. PVT was completely recanalized with single oral anticoagulant therapy rivaroxaban as initial low molecular weight heparin, enoxaparin administration caused reversible pancytopenia and there is a concern for bleeding and regular monitoring of INR with warfarin in this patient. Keywords: Portal vein thrombosis; Chronic liver disease; Protein S deficiency; Oral anticoagulant; Portal hypertension; Thrombolysis

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Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency

PEDIATRICS ◽

10.1542/peds.2013-1156 ◽

2013 ◽

Vol 132 (5) ◽

pp. e1435-e1439 ◽

Cited By ~ 29

Author(s):

Ida Martinelli ◽

Paolo Bucciarelli ◽

Andrea Artoni ◽

Emilio F. Fossali ◽

Serena M. Passamonti ◽

...

Keyword(s):

Protein S ◽

Protein S Deficiency ◽

Anticoagulant Treatment ◽

S Deficiency

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Faculty Opinions recommendation of Anticoagulant treatment with rivaroxaban in severe protein S deficiency.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718151570.793485948 ◽

2013 ◽

Author(s):

Gili Kenet

Keyword(s):

Protein S ◽

Protein S Deficiency ◽

Anticoagulant Treatment ◽

S Deficiency

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Oral Anticoagulant Treatment with and without Aspirin

Thrombosis and Haemostasis ◽

10.1055/s-0038-1642729 ◽

1995 ◽

Vol 74 (01) ◽

pp. 506-510 ◽

Cited By ~ 11

Author(s):

Raul Altman ◽

Jorge Rouvier ◽

Enrique Gurfinkel

Keyword(s):

Oral Anticoagulant ◽

Anticoagulant Treatment ◽

Oral Anticoagulant Treatment

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

Thrombosis and Haemostasis ◽

10.1055/s-0038-1642558 ◽

1988 ◽

Vol 59 (01) ◽

pp. 018-022 ◽

Cited By ~ 139

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.

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Severe Arterial Cerebral Thrombosis in a Patient with Protein S Deficiency (Moderately Reduced Total and Markedly Reduced Free Protein S): A Family Study

Thrombosis and Haemostasis ◽

10.1055/s-0038-1646544 ◽

1989 ◽

Vol 61 (01) ◽

pp. 144-147 ◽

Cited By ~ 47

Author(s):

A Girolami ◽

P Simioni ◽

A R Lazzaro ◽

I Cordiano

Keyword(s):

Arterial Thrombosis ◽

Protein C ◽

Protein S ◽

Protein S Deficiency ◽

Protein C Deficiency ◽

Free Protein ◽

Her Family ◽

S Deficiency ◽

Increased Risk ◽

Patient Reported

SummaryDeficiency of protein S has been associated with an increased risk of thrombotic disease as already shown for protein C deficiency. Deficiencies of any of these two proteins predispose to venous thrombosis but have been only rarely associated with arterial thrombosis.In this study we describe a case of severe cerebral arterial thrombosis in a 44-year old woman with protein S deficiency. The defect was characterized by moderately reduced levels of total and markedly reduced levels of free protein S. C4b-bp level was normal. Protein C, AT III and routine coagulation tests were within the normal limits.In her family two other members showed the same defect. All the affected members had venous thrombotic manifestations, two of them at a relatively young age. No other risk factors for thrombotic episodes were present in the family members. The patient reported was treated with ASA and dipyridamole and so far there were no relapses.

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