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Clin Appl Thromb Hemost 2021 Jan-Dec;27:10760296211033908

Department of Clinical Chemistry and Laboratory Medicine, Kyushu University School of Medicine, Fukuoka, Japan.

The quantitative assay of protein S can help in rapidly identifying carriers of abnormal protein S molecules through a simple procedure (by determining the total protein S mass, total protein S activity, and protein S-specific activity in blood), without genetic testing. To clarify the relationship between venous thromboembolism (VTE) and protein S-specific activity, and its role in the diagnosis of thrombosis in Japanese persons, the protein S-specific activity was measured and compared between patients with thrombosis and healthy individuals. The protein S-specific activity of each participant was calculated from the ratio of total protein S activity to total protein S antigen level. Read More

Thromb J 2021 Jul 17;19(1):50. Epub 2021 Jul 17.

Department of Internal Medicine, Division of Cardiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 123, Ta Pei Road, Niao Sung District, Kaohsiung City, 83301, Taiwan.

Background: Non-vitamin K antagonist oral anticoagulants (NOACs) are as effective and safe as warfarin for thromboembolic prevention and treatment. The efficacy of NOACs lacks evidence from large and randomized studies in patients with inherited severe thrombophilia, including protein S deficiency. Further, some concerns still exist regarding the relative efficacy of edoxaban in preventing arterial thromboembolism in patients with normal to high creatinine clearance (CrCl). Read More

Res Pract Thromb Haemost 2021 Jul 28;5(5):e12559. Epub 2021 Jun 28.

Department of Hematology and Oncology University Cancer Center Hamburg (UCCH) University Medical Center Eppendorf Hamburg Germany.

Autoimmune protein S (PS) deficiency is a highly thrombotic, potentially life-threatening disorder. Its pathophysiological relevance in the context of primary antiphospholipid syndrome (APS) is unclear. Here, we report the case of a 76-year-old woman, who presented with a painful reticular skin erythema caused by microvascular thromboses. Read More

J Obstet Gynaecol Res 2021 Jun 24. Epub 2021 Jun 24.

The Study Group for Hereditary Thrombophilia, Research on Blood Coagulation Abnormalities, Research Program on Rare and Intractable Diseases, The Ministry of Health, Labour and Welfare Science Research Grants, Tokyo, Japan.

Hereditary thrombophilia is a condition in which individuals are susceptible to the formation of thrombi due to a hereditary deficiency in anticoagulant factors, antithrombin (AT), protein C (PC), or protein S (PS). Many Japanese thrombophilia patients have PS deficiency, especially PS p.K196E (also called as PS Tokushima), which is exclusive to the Japanese population, and thrombosis sometimes occurs during pregnancy. Read More

Indian J Med Res 2020 Dec;152(6):584-594

Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Background & Objectives: Vegetarianism may result in low vitamin B12 and acquired hyperhomocysteinemia leading to thrombotic conditions such as cerebral venous sinus thrombosis (CVST). The clinico-radiological presentation and outcome of patients with hyperhomocysteinemia may be different from those without, but there is a paucity of information. This study was undertaken to find out the relationship of homocysteine (Hcy) with vitamin B12, folic acid and methyltetrahydrofolate reductase (MTHFR) mutation in the patients with CVST, and compare clinico-radiological severity and outcome of patients with and without hyperhomocysteinemia. Read More

Oral Surg Oral Med Oral Pathol Oral Radiol 2021 Mar 6. Epub 2021 Mar 6.

Department of Oral and Maxillofacial Surgery, University Hospitals Leuven, Leuven, Belgium.

Objective: The aim of this study was to analyze patients with thrombophilia who underwent oral and/or maxillofacial surgery at our center.

Study Design: We performed a retrospective analysis of patients with hereditary or acquired thrombophilia who had undergone oral/maxillofacial surgery between January 1, 2000 and December 31, 2019. Data regarding demographic and patient characteristics, surgical treatment modalities, antithrombotic therapies, and complications were analyzed. Read More

Clin Appl Thromb Hemost 2021 Jan-Dec;27:10760296211016550

Department of Medicine I, Klinikum Heidenheim, Heidenheim, Germany.

The management of pregnant women at increased risk of thromboembolic/other vascular events is still a matter of debate. In a single-center, retrospective, observational trial, we analyzed the safety and efficacy of prophylactic anticoagulation with certoparin in pregnant women at intermediate- or high-risk by EThIG criteria of thromboembolic/other vascular events. Subcutaneous certoparin 8,000 IU once daily was administered immediately after pregnancy confirmation and continued for 6 weeks postpartum. Read More

Ann Clin Lab Sci 2021 Mar;51(2):206-212

Department of Clinical Laboratory Science, Division of Health Sciences, Graduate School of Medical Science, Kanazawa University

Objective: Protein S(PS) activity, especially PS-specific activity calculated by total PS activity (tPSAct) divided by total PS antigen (tPSAg), is important in the diagnosis of hereditary PS deficiency (PSD). The cleavage of PS at a thrombin-sensitive region (TSR) by proteases reduces the anticoagulant activity of PS. Therefore, we investigated the effect of sample processing and storage on tPSAct and PS cleavage. Read More

Ghana Med J 2020 Dec;54(4):269-273

National Reconstructive Plastic Surgery and Burns Centre, Korle Bu Teaching Hospital, Accra, Ghana.

Warfarin induced skin necrosis is a rare debilitating and, in some cases, life-threatening complication. A 47-year-old male on life-long anticoagulation omits his medication and develops extensive skin necrosis of the left leg complicated by acute renal failure three days after restarting warfarin. Investigations reveal possible Protein S deficiency which is known to be a predisposing condition. Read More

Cureus 2021 Mar 13;13(3):e13866. Epub 2021 Mar 13.

Internal Medicine, St. Luke's University Health Network, Easton, USA.

Protein S is a potent anticoagulant that downregulates thrombin formation and is a vitamin K-dependent glycoprotein which is primarily synthesized in the liver. A deficiency in this protein or decreased activity, as seen in hereditary protein S deficiency, can lead to life-threatening thrombosis. Hereditary protein S deficiency is a rare disease as listed by the National Organization for Rare Disorders (NORD). Read More

Zhonghua Jie He He Hu Xi Za Zhi 2021 Apr;44(4):360-364

Center of Laboratory Medicine, the First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325015, China.

To observe the clinical feature of familiar hereditary protein S deficiency, and to explore the related gene mutation. The blood samples were obtained from the proband and the family memebers(3 generations,6 persons). gene of the proband and the family members was analyzed. Read More

Indian J Anaesth 2021 Mar 13;65(3):267-268. Epub 2021 Mar 13.

Department of Anaesthesiology, Kokilaben Dhirubhai Ambani Hospital and Research Institute, Mumbai, Maharashtra, India.

World J Clin Cases 2021 Mar;9(8):2008-2014

Department of Hematology, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China.

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by systemic involvement and multiple autoantibodies in the serum. Patients with protein C (PC) and protein S (PS) deficiency are prone to thrombosis. In contrast, patients with primary hyperfibrino-lysis tend to bleed. Read More

Eur J Clin Invest 2021 May 25;51(5):e13546. Epub 2021 Mar 25.

Servicio de Hematología y Oncología Médica, Centro Regional de Hemodonación, Hospital Universitario Morales Meseguer, Universidad de Murcia, IMIB-Arrixaca, CIBERER, Murcia, Spain.

Cardiology 2021;146(3):375-383. Epub 2021 Feb 12.

Department of Family Medicine, Clalit Health Services, Tel-Aviv, Israel.

Background: Percutaneous device closure was shown to effectively prevent recurrent strokes in patients with patent foramen ovale (PFO). Whether this protective effect is relevant for patients with hypercoagulable states (HCSs) is unknown as they were not represented in prior studies.

Methods: Data on 136 consecutive patients with a PFO and clinically significant HCS were retrospectively collected. Read More

Res Pract Thromb Haemost 2021 Jan 27;5(1):179-186. Epub 2020 Nov 27.

Department of Molecular Pathogenesis National Cerebral and Cardiovascular Center Suita Japan.

Background: Genetic deficiencies of antithrombin (AT), protein C (PC), and protein S (PS) are risk factors for venous thromboembolism. In the general population, the prevalence of heterozygous deficiency of AT, PC, and PS are reported as approximately 0.02%-0. Read More

Neurosciences (Riyadh) 2021 Jan;26(1):15-20

From the Faculty of Medicine (Daghriri, Alrajhai, Aburasain, Ateya), Department of Family and Community Medicine (Gosadi), Faculty of Medicine, Jazan University, and Department of Neurology (Hakami), King Fahad Central Hospital, Jazan, Kingdom of Saudi Arabia.

Objectives: To assess cerebral venous thrombosis risk factors, and associated clinical outcomes in Jazan region, Kingdom of Saudi Arabia.

Methods: This study is a retrospective review of the medical records of patients diagnosed with cerebral venous thrombosis and admitted to King Fahad Central Hospital in Jazan between 2010 and 2019. Data concerning socio-demographics, clinical features, risk factors, laboratory, and imaging investigations were retrieved. Read More

Neurol Ther 2021 Jun 30;10(1):401-406. Epub 2021 Jan 30.

Isfahan Neurosciences Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.

Protein S deficiency is a thrombophilia associated with an increased risk of thromboembolism. Previous studies have shown its role as a predisposing factor for venous thromboembolism, but its role in recurrent arterial ischemic stroke remains uncertain. Here we report a patient with recurrent ischemic stroke due to protein S deficiency. Read More

Asian Pac J Cancer Prev 2021 Jan 1;22(1):267-275. Epub 2021 Jan 1.

Departments of Clinical Pathology, National Liver Institute, Menoufia University, Egypt.

Background: Portal vein thrombosis (PVT) might be a catastrophic event complicating liver cirrhosis and hepatocellular carcinoma (HCC).

Aim: role of JAK2 RS V617F mutation as a risk factor for PVT development in liver cirrhosis and HCC.

Methods: A case control study conducted on 100 PVT patients (76 HCC and 24 liver cirrhosis) additionally, 100 healthy individuals used as a control group. Read More

Eur Rev Med Pharmacol Sci 2021 01;25(1):353-361

Department of Geriatric Medicine, Qilu Hospital of Shandong University, Jinan, China.

Objective: To explore the clinical and prognostic features of CVT caused by PROS1 gene mutations and to provide clinical experience for new oral anticoagulants, such as rivaroxaban, in the treatment of CVT with a high risk of thrombosis.

Patients And Methods: The CVT patient's clinical symptoms were described, and the brain imaging and blood coagulation tests were performed to confirm the diagnosis of CVT. The patient's family members were recruited to receive blood coagulation tests and ultrasonic examination of lower limb vessels. Read More

Taiwan J Obstet Gynecol 2021 Jan;60(1):148-151

Department of Obstetrics and Gynecology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan. Electronic address:

Objective: The risk of venous thromboembolism in pregnancies increased in ovarian hyperstimulation syndrome (OHSS) after assisted reproductive technologies (ART). We present a rare case with protein S deficiency receiving ART treatment with OHSS, following right neck venous thromboembolism.

Case Report: A 34-year-old women with primary infertility underwent IVF treatment and presented with OHSS. Read More

Neurol Sci 2021 Jan 20. Epub 2021 Jan 20.

Institute of Neurology, Department of Applied Clinical Sciences and Biotechnology, University of L'Aquila, L'Aquila, Italy.

Background: The association between thrombophilic alterations, migraine, and vascular events has been broadly investigated but not been completely clarified.

Methods: In this cross-sectional, case-control study, we included consecutive outpatients diagnosed with migraine referring to a tertiary headache center. Migraine patients were matched to headache-free control subjects. Read More

Int J Hematol 2021 Apr 8;113(4):530-536. Epub 2021 Jan 8.

Department of Clinical Chemistry and Laboratory Medicine, Kyushu University Hospital, Fukuoka, 812-0054, Japan.

Patients with congenital protein S (PS) deficiency show a hereditary predisposition for thrombosis, and PS deficiency is prevalent among Japanese populations. Diagnosis is based on symptoms of thrombosis and reduced PS activity. Three reagents that use different measurement principles for determining PS activity are available in Japan. Read More

J Neurol 2021 Mar 8;268(3):1110-1113. Epub 2021 Jan 8.

Department of Neurology, Seoul National University College of Medicine, Seoul, Republic of Korea.

J Blood Med 2020 30;11:515-524. Epub 2020 Dec 30.

Department of Internal Medicine, Helwan University, Helwan, Egypt.

Background And Objectives: Budd-Chiari syndrome (BCS) is a rare disorder caused by obstruction to hepatic venous outflow. It affects all races, usually during the third or fourth decade of life. Higher prevalence had being evident in developing countries. Read More

J Thromb Haemost 2021 01;19(1):281-286

Department of Pathology, University of New Mexico, TriCore Reference Laboratories, Albuquerque, NM, USA.

Hereditary deficiencies of protein S (PS) increase the risk of thrombosis. However, assessing the plasma levels of PS is complicated by its manifold physiological interactions, while the large inter-individual variability makes it problematic to establish reliable cut-off values. PS has multiple physiological functions, with only two appearing to have significant anticoagulant properties: the activated protein C (APC) and tissue factor pathway inhibitor alpha (TFPIα) cofactor activities. Read More

J Thromb Haemost 2021 01;19(1):68-74

Department of Molecular and Cellular Hemostasis, Sanquin Research, Amsterdam, The Netherlands.

Hereditary deficiencies of protein S (PS) increase the risk of venous thrombosis; however, assessing the plasma levels of PS can be difficult because of its complex physiological interactions in plasma, sample-related preanalytical variables, and numerous acquired disease processes. Reliable laboratory assays are essential for accurate evaluation of PS when diagnosing a congenital deficiency based on the plasma phenotype alone. This report presents the current evidence-based recommendations for clinical PS assays as well as when to test for PS abnormalities. Read More

Int J Lab Hematol 2021 04 20;43(2):146-147. Epub 2020 Dec 20.

Department of Laboratory Medicine, Ghent University Hospital, Ghent, Belgium.

J Appl Lab Med 2021 Jan;6(1):101-112

Department of Neurology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.

Background: Prothrombotic factors have been correlated with vascular events in young patients, with recurrent strokes, and with venous thromboembolisms. However, their prevalence in adult strokes, in healthy populations, and in specific ethnic groups is not well defined. We investigated the association of prothrombotic factors with strokes in a South Indian Tamil population. Read More

Blood Adv 2020 12;4(23):5958-5965

State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital.

The prevalence and distribution of congenital thrombophilia is still unclear in patients with pulmonary embolism (PE). We aimed to determine the prevalence and clinical characteristics of congenital thrombophilia in PE patients and their subsequent outcomes. A prospective observational study was conducted from May 2013 to June 2018. Read More