Research & Literature

Idarucizumab for Dabigatran Reversal.

Pollack CV, Reilly PA, Eikelboom J, et al.

The New England journal of medicine 2015; (373(6)):511-20 doi:10.1056/NEJMoa1502000.

Profile of betrixaban and its potential in the prevention and treatment of venous thromboembolism.

Chan NC, Bhagirath V, Eikelboom JW

Vascular health and risk management 2015; (11()):343-51 doi:10.2147/VHRM.S63060.

PROS1 genotype phenotype relationships in a large cohort of adults with suspicion of inherited quantitative protein S deficiency.

Alhenc-Gelas M, Plu-Bureau G, Horellou MH, et al.

Thrombosis and haemostasis 2016; (115(3)):570-9 doi:10.1160/TH15-05-0391.

Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report.

Kearon C, Akl EA, Ornelas J, et al.

Chest 2016; (149(2)):315-352 doi:10.1016/j.chest.2015.11.026.

Combined oral contraceptives, thrombophilia and the risk of venous thromboembolism: a systematic review and meta-analysis.

van Vlijmen EF, Wiewel-Verschueren S, Monster TB, Meijer K

Journal of thrombosis and haemostasis : JTH 2016; (14(7)):1393-403 doi:10.1111/jth.13349.

[Hereditary thrombophilia testing and its therapeutic impact on venous thromboembolism disease: Results from a retrospective single-center study of 162 patients].

Allain JS, Gueret P, Le Gallou T, et al.

La Revue de medecine interne 2016; (37(10)):661-666 doi:10.1016/j.revmed.2016.01.001.

Deep vein thrombosis and pulmonary embolism.

Di Nisio M, van Es N, Büller HR

Lancet (London, England) 2016; (388(10063)):3060-3073 doi:10.1016/S0140-6736(16)30514-1.

A novel PROS1 mutation, c.74dupA, was identified in a protein S deficiency family.

Zhang Y, Yang H, Chen Q, et al.

Thrombosis research 2016; (148()):125-127 doi:10.1016/j.thromres.2016.11.003.

Thromboelastography (TEG®) demonstrates that tinzaparin 4500 international units has no detectable anticoagulant activity after caesarean section.

Griffiths S, Woo C, Mansoubi V, et al.

International journal of obstetric anesthesia 2017; (29()):50-56 doi:10.1016/j.ijoa.2016.10.001.

Non-vitamin K antagonist oral anticoagulants in patients with severe inherited thrombophilia: a series of 33 patients.

Undas A, Goralczyk T

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2017; (28(6)):438-442 doi:10.1097/MBC.0000000000000613.

Gene analysis of six cases of congenital protein S deficiency and functional analysis of protein S mutations (A139V, C449F, R451Q, C475F, A525V and D599TfsTer13).

Taniguchi F, Morishita E, Sekiya A, et al.

Thrombosis research 2017; (151()):8-16 doi:10.1016/j.thromres.2016.12.018.

Inherited Thrombophilia in Pediatric Venous Thromboembolic Disease: Why and Who to Test.

van Ommen CH, Nowak-Göttl U

Frontiers in pediatrics 2017; (5()):50 doi:10.3389/fped.2017.00050.

Residual Enoxaparin Activity, Anti-Xa Levels, and Concerns About the American Society of Regional Anesthesia and Pain Medicine Anticoagulation Guidelines.

Henshaw DS, Turner JD, Forest DJ, et al.

Regional anesthesia and pain medicine 2017; (42(4)):432-436 doi:10.1097/AAP.0000000000000617.

New developments in anticoagulants: Past, present and future.

Weitz JI, Harenberg J

Thrombosis and haemostasis 2017; (117(7)):1283-1288 doi:10.1160/TH16-10-0807.

Vitamin K-Dependent Protein S: Beyond the Protein C Pathway.

Dahlbäck B

Seminars in thrombosis and hemostasis 2018; (44(2)):176-184 doi:10.1055/s-0037-1604092.

Challenges of Anticoagulation Therapy in Pregnancy.

Fogerty AE

Current treatment options in cardiovascular medicine 2017; (19(10)):76 doi:10.1007/s11936-017-0575-x.

Rivaroxaban Causes Missed Diagnosis of Protein S Deficiency but Not of Activated Protein C Resistance (Factor V Leiden).

Maryamchik E, Rosenbaum MW, Van Cott EM

Archives of pathology & laboratory medicine 2018; (142(1)):70-74 doi:10.5858/arpa.2016-0616-OA.

Pregnancy, thrombophilia, and the risk of a first venous thrombosis: systematic review and bayesian meta-analysis.

Croles FN, Nasserinejad K, Duvekot JJ, et al.

BMJ (Clinical research ed.) 2017; (359()):j4452 doi:10.1136/bmj.j4452.

Edoxaban improves acute venous thromboembolism while preserving protein C and protein S levels.

Yamazaki H, Yagi S, Torii Y, et al.

Journal of cardiology 2018; (71(3)):305-309 doi:10.1016/j.jjcc.2017.09.009.

Overview of venous thromboembolism.

Phillippe HM

The American journal of managed care 2017; (23(20 Suppl)):S376-S382.

Thrombophilia testing in children: What and when should be tested?

Nowak-Göttl U, van Ommen H, Kenet G

Thrombosis research 2018; (164()):75-78 doi:10.1016/j.thromres.2018.02.136.

Management of Venous Thromboembolism in Pregnancy.

Fogerty AE

Current treatment options in cardiovascular medicine 2018; (20(8)):69 doi:10.1007/s11936-018-0658-3.

Biomarkers, menopausal hormone therapy and risk of venous thrombosis: The Women's Health Initiative.

Cushman M, Larson JC, Rosendaal FR, et al.

Research and practice in thrombosis and haemostasis 2018; (2(2)):310-319 doi:10.1002/rth2.12100.

Phlegmasia Cerulea Dolens with Compartment Syndrome.

Chaochankit W, Akaraborworn O

Annals of vascular diseases 2018; (11(3)):355-357 doi:10.3400/avd.cr.18-00030.

Clinical Manifestation and Mutation Spectrum of 53 Unrelated Pedigrees with Protein S Deficiency in China.

Li L, Wu X, Wu W, et al.

Thrombosis and haemostasis 2019; (119(3)):449-460 doi:10.1055/s-0038-1677031.

Laboratory Diagnostics in Thrombophilia.

Linnemann B, Hart C

Hamostaseologie 2019; (39(1)):49-61 doi:10.1055/s-0039-1677840.

New guidelines from the Thrombosis and Haemostasis Society of Australia and New Zealand for the diagnosis and management of venous thromboembolism.

Tran HA, Gibbs H, Merriman E, et al.

The Medical journal of Australia 2019; (210(5)):227-235 doi:10.5694/mja2.50004.

The roles of factor Va and protein S in formation of the activated protein C/protein S/factor Va inactivation complex.

Gierula M, Salles-Crawley II, Santamaria S, et al.

Journal of thrombosis and haemostasis : JTH 2019; (17(12)):2056-2068 doi:10.1111/jth.14594.

Plasma phenotypes of protein S Lys196Glu and protein C Lys193del variants prevalent among young Japanese women.

Noguchi K, Nakazono E, Tsuda T, et al.

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2019; (30(8)):393-400 doi:10.1097/MBC.0000000000000854.

Acute treatment of venous thromboembolism.

Becattini C, Agnelli G

Blood 2020; (135(5)):305-316 doi:10.1182/blood.2019001881.

How Do I Reverse Oral and Parenteral Anticoagulants?

Koscielny J, Rutkauskaite E, Sucker C, von Heymann C

Hamostaseologie 2020; (40(2)):201-213 doi:10.1055/a-1113-0557.

Multimodal approach of venous recanalization in patients with a critical limb ischemia due to phlegmasia cerulea dolens: A case series of 17 patients in a single center.

Mühlberger D, Mumme A, Stücker M, et al.

Phlebology 2020; (35(9)):701-705 doi:10.1177/0268355520935744.

Comprehensive review of the impact of direct oral anticoagulants on thrombophilia diagnostic tests: Practical recommendations for the laboratory.

Siriez R, Dogné JM, Gosselin R, et al.

International journal of laboratory hematology 2021; (43(1)):7-20 doi:10.1111/ijlh.13342.

American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism.

Ortel TL, Neumann I, Ageno W, et al.

Blood advances 2020; (4(19)):4693-4738 doi:10.1182/bloodadvances.2020001830.

Direct Oral Anticoagulants in Select Patients With Hypercoagulable Disorders.

Valanejad SM, Davis KA

The Annals of pharmacotherapy 2021; (55(7)):891-901 doi:10.1177/1060028020968551.

Incidence of VTE in asymptomatic children with deficiencies of antithrombin, protein C, and protein S: a prospective cohort study.

Tormene D, Campello E, Simion C, et al.

Blood advances 2020; (4(21)):5442-5448 doi:10.1182/bloodadvances.2020002781.

Anticoagulation for Patients with Venous Thromboembolism: When is Extended Treatment Required?

Weitz JI, Prandoni P, Verhamme P

TH open : companion journal to thrombosis and haemostasis 2020; (4(4)):e446-e456 doi:10.1055/s-0040-1721735.

Recommendations for clinical laboratory testing for protein S deficiency: Communication from the SSC committee plasma coagulation inhibitors of the ISTH.

Marlar RA, Gausman JN, Tsuda H, et al.

Journal of thrombosis and haemostasis : JTH 2021; (19(1)):68-74 doi:10.1111/jth.15109.

Pleiotropic anticoagulant functions of protein S, consequences for the clinical laboratory. Communication from the SSC of the ISTH.

Brinkman HJM, Ahnström J, Castoldi E, et al.

Journal of thrombosis and haemostasis : JTH 2021; (19(1)):281-286 doi:10.1111/jth.15108.

Venous thromboembolism.

Khan F, Tritschler T, Kahn SR, Rodger MA

Lancet (London, England) 2021; (398(10294)):64-77 doi:10.1016/S0140-6736(20)32658-1.

Protein S: function, regulation, and clinical perspectives.

Majumder R, Nguyen T

Current opinion in hematology 2021; (28(5)):339-344 doi:10.1097/MOH.0000000000000663.

Effect on Plasma Protein S Activity in Patients Receiving the Factor Xa Inhibitors.

Terakami T, Nagaya S, Hayashi K, et al.

Journal of atherosclerosis and thrombosis 2022; (29(7)):1059-1068 doi:10.5551/jat.62951.

Genetic Variants in the Protein S ( PROS1 ) Gene and Protein S Deficiency in a Danish Population.

Larsen OH, Kjaergaard AD, Hvas AM, Nissen PH

TH open : companion journal to thrombosis and haemostasis 2021; (5(4)):e479-e488 doi:10.1055/s-0041-1736636.

Recurrent hematuria and painful necrotic purpura induced by acquired Protein S deficiency associated with monoclonal immunoglobulin.

Tang W, Guo Y

Journal of thrombosis and thrombolysis 2022; (54(1)):156-161 doi:10.1007/s11239-022-02632-6.

[A family of hereditary protein S deficiency with the onset of pulmonary embolism and literature review].

Wei CJ, Guo CY, Li QR, Ye LP

Zhonghua er ke za zhi = Chinese journal of pediatrics 2022; (60(2)):134-138 doi:10.3760/cma.j.cn112140-20210915-00797.

Inherited Thrombophilia in the Era of Direct Oral Anticoagulants.

Khider L, Gendron N, Mauge L

International journal of molecular sciences 2022; (23(3)) doi:10.3390/ijms23031821.

Antithrombotic potential of a single-domain antibody enhancing the activated protein C-cofactor activity of protein S.

Sedzro JC, Adam F, Auditeau C, et al.

Journal of thrombosis and haemostasis : JTH 2022; (20(7)):1653-1664 doi:10.1111/jth.15736.

"The Great Masquerader": An Interesting Case Series of Pulmonary Thromboembolism.

Sritharan SB, Raj CP, Ta V, et al.

Cureus 2022; (14(12)):e32330 doi:10.7759/cureus.32330.

Onset of labor and use of analgesia in women using thromboprophylaxis with 2 doses of low-molecular-weight heparin: insights from the Highlow study.

Bistervels IM, Wiegers HMG, Áinle FN, et al.

Journal of thrombosis and haemostasis : JTH 2023; (21(1)):57-67 doi:10.1016/j.jtha.2022.11.004.

American Society of Hematology 2023 guidelines for management of venous thromboembolism: thrombophilia testing.

Middeldorp S, Nieuwlaat R, Baumann Kreuziger L, et al.

Blood advances 2023; (7(22)):7101-7138 doi:10.1182/bloodadvances.2023010177.

Clinical Relevance of Preclinical and Clinical Studies of Four-Factor Prothrombin Complex Concentrate for Treatment of Bleeding Related to Direct Oral Anticoagulants.

Sarode R, Welsby IJ, Hoffman M

Annals of emergency medicine 2023; (82(3)):341-361 doi:10.1016/j.annemergmed.2023.04.015.

Neutrophil extracellular traps mediate deep vein thrombosis: from mechanism to therapy.

Yao M, Ma J, Wu D, et al.

Frontiers in immunology 2023; (14()):1198952 doi:10.3389/fimmu.2023.1198952.

Analysis of PROS1 mutations and clinical characteristics in three Chinese families with hereditary protein S deficiency.

Xu F, Zhou X, Jin Y, et al.

Annals of hematology 2024; (103(2)):653-662 doi:10.1007/s00277-023-05607-6.

Thrombophilia Screening: Not So Straightforward.

Moore GW

Seminars in thrombosis and hemostasis 2024; (50(8)):1131-1152 doi:10.1055/s-0044-1786807.

Venous thromboembolism risk in adults with hereditary thrombophilia: a systematic review and meta-analysis.

Alnor AB, Gils C, Vinholt PJ

Annals of hematology 2024; (103(10)):4285-4294 doi:10.1007/s00277-024-05926-2.

The use of direct oral anticoagulants in the secondary prevention of venous thromboembolism in patients with severe thrombophilia: communication from the ISTH SSC Subcommittee on Physiological Anticoagulants and Thrombophilia.

Kovac M, Ignjatovic V, Orlando C, et al.

Journal of thrombosis and haemostasis : JTH 2024; (22(11)):3322-3329 doi:10.1016/j.jtha.2024.08.006.

Impact of thrombophilia on venous thromboembolism management.

Emmerich J, Zuily S, Gouin-Thibault I, et al.

Presse medicale (Paris, France : 1983) 2024; (53(4)):104247 doi:10.1016/j.lpm.2024.104247.

Two patients with protein S deficiency and cerebral venous sinus thrombosis: nonsense mutations of the PROS1 gene may account for these deficiencies.

Hou L, Chen X, Xie H, et al.

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2025; (36(2)):51-57 doi:10.1097/MBC.0000000000001343.

Population-Scale Studies of Protein S Abnormalities and Thrombosis.

Chaudhry SA, Haj AK, Ryu J, et al.

JAMA 2025; (333(16)):1423-1432 doi:10.1001/jama.2025.0155.

Phlegmasia Cerulea Dolens in a 21-Year-Old Female Patient Following Depo-Provera Injection: A Case Report.

Ansong S, Meany J, Umerah C, Okafor C

Cureus 2025; (17(2)):e79426 doi:10.7759/cureus.79426.

Thrombosis risk with the use of hormonal contraception among women with thrombophilia: An updated systematic review.

Tepper NK, Nguyen AT, Curtis KM, et al.

Contraception 2026; (159()):110943 doi:10.1016/j.contraception.2025.110943.

Diagnosing protein S deficiency - Navigating challenges.

Hansen RS, Nissen PH, Larsen JB, Bor MV

Clinical biochemistry 2025; (138()):110950 doi:10.1016/j.clinbiochem.2025.110950.

Diagnosis of Venous Thromboembolism.

Canas A, Epstein J, Chen T, Dunn AS

The Medical clinics of North America 2025; (109(4)):787-801 doi:10.1016/j.mcna.2025.01.006.

Clinical utility and costs of inpatient hereditary thrombophilia testing following acute VTE: A 5-Year retrospective study.

Munda E, Rhoades R

Journal of thrombosis and thrombolysis 2026; (59(2)):462-471 doi:10.1007/s11239-025-03183-2.

Inherited thrombophilias: Genetics and testing considerations.

Tinkle MB

Journal of the American Association of Nurse Practitioners 2026; (38(1)):2-7 doi:10.1097/JXX.0000000000001216.

Management of patients with protein s deficiency: focus on clinical course and direct oral anticoagulants.

Altamura N, Di Girolamo FG, Pradella P, et al.

Thrombosis journal 2026; (24(1)).

An interactive human PROS1 variants database provides novel insights into the genetics and phenotypes of inherited protein S deficiency.

Hou Z, Liu F, Dong S, et al.

Journal of thrombosis and haemostasis : JTH 2026; (24(7)):2579-2592 doi:10.1016/j.jtha.2026.04.004.

Identification of novel PROS1 variants through systematic analysis of patients with suspected hereditary protein S deficiency.

Bartylla MM, Achenbach S, Hackstein H, Schneider S

Research and practice in thrombosis and haemostasis 2026; (10(3)):103432 doi:10.1016/j.rpth.2026.103432.

Physiological anticoagulant deficiencies: pathogenesis, diagnosis, and clinical implications.

Mayger K, Orlando C, Hunt BJ

Journal of thrombosis and haemostasis : JTH 2026; (24(9)):3116-3127 doi:10.1016/j.jtha.2026.06.020.