Protein S Deficiency and Arterial Thromboembolism: A Case Report and Review of the Literature

Background/Aim. After introduction of carefull selection procedure for blood donors and the implemention of highly sensitive screening tests for transfusion transmissible infections (TTI), blood is very save product concerning TTI. Nevertheless, because of the ?window? period for the pathogen that are testing and the emergence of new pathogens, the risk stil persists. Implementation of pathogen reduction technology (PRT) provides a proactive approach to improve blood safety. By damaging nucleic acids, PRT selectively inactivates pathogens and leucocytes. However, during the process, plasma proteins are, also, damaged in some extent. The goal of this study is to conclude if there is the difference in the effect of PRT on Protein S and ?2-antiplasmin regarding the time of inactivation: inactivation immediately after plasma separation from whole blood (before freezing) versus inactivation after freezing/thawing. Methods. The voluntary donors' blood is taken into quadruple bag system, centrifuged and separated into blood products. Control's group plasma is inactivated by the Mirasol PRT system and frozen. Experimental's group plasma is frozen and, after four months, thawed and inactivated. Protein S and ?2-antiplasmin activity are examined in samples after separation, inactivation and thawing. Results. Analysing Protein S and ?2-antiplasmin activity, no statistically significant difference is found between the initial samples. The trend of reduction of protein's activity after inactivation and freezing/thawing is present in both groups but without statistically significant intergroup difference. Conclusion. Reduction in Protein S and ?2-antiplasmin's activity after immediate - before freezing and afterwards - after freezing/thawing inactivation does not show a statistically significant difference, making stored plasma unites suitable for safe and efficient inactivation directly before clinical use and according to patient's blood type.

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“…Protein S deficiency leads to risk of venous thrombosis but could be, as well, associated with arterial thrombotic events 17 .…”

Section: Discussionmentioning

confidence: 99%

Influence of riboflavin and ultraviolet-light treatment on plasma proteins – protein S and alpha 2-antiplasmin – in relation to the time of administration

et al. 2022

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“…Hereditary protein S deficiency is autosomal dominant in inheritance. It is caused by multiple mutations involving the PROS1 gene [ 9 , 12 - 14 ], with heterozygous mutations resulting in mild disease and homozygous mutations causing severe disease [ 12 ]. Hereditary protein S deficiency is divided into three types depending on protein S antigen and activity levels.…”

Section: Discussionmentioning

confidence: 99%

A Rare Thrombophilic Occurrence: Dural Venous Sinus Thrombosis in a Patient with Significant Family History of Protein S Deficiency

Amaratunga¹,

Kamau²,

Ernst³

et al. 2021

Cureus

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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). It is known to cause venous as well as arterial thromboembolic events commonly occurring in the deep leg and pelvic veins. Dural venous sinus thrombosis is a rare consequence of protein S deficiency and is associated with a risk of increased morbidity and mortality. We report a case of dural venous sinus thrombosis in a patient with a family history of protein S deficiency in nine family members. A 53-year-old female presented to the ED with a three-day history of persistent left-sided headache, left facial numbness with tingling, and photophobia. She denied any visual disturbances, slurring of speech, and/or unilateral weakness. Some 10 years prior to this episode, she was placed on warfarin therapy for deep vein thrombosis (DVT) of lower extremity, but she discontinued it after three years of treatment without consulting her treating physician. She was taking oral contraceptive pills (OCPs) for two years and discontinued one month ago. She has nine family members with protein S deficiency, but the patient was never screened for a hypercoagulable state. On admission, her vital signs were within normal limits. Pupils were round and reactive to light, neck was supple, there was a sensory deficit for pinprick on the left V2-V3 distribution, and remainder of the cranial nerves and neurologic examination was unremarkable. CT scan of the head demonstrated a hyper-density within the left transverse and sigmoid sinus suspicious for dural venous sinus thrombosis. This was confirmed by CT angiogram showing a filling defect throughout the transverse sinus and sigmoid sinus extending below the jugular bulb into the superior aspect of the jugular vein. Intravenous heparin and warfarin were initiated. As the patient had severe trypanophobia and IV heparin required frequent activated partial thromboplastin time (APTT) monitoring, this was later changed to subcutaneous low-molecular-weight heparin and warfarin. Subsequent thrombosis panel showed a reduced protein S activity of 15% and low levels of total and free protein S antigens. She was discharged home with life-long warfarin therapy. In conclusion, cerebral dural venous sinus thrombosis is a rare and potentially life-threatening condition that can be seen in hereditary protein S deficiency. A high degree of suspicion in young females with worsening headache and neurologic signs and symptoms will help with timely diagnosis and management avoiding serious consequences. In a patient with a family history of thrombophilia, as seen in our patient, screening is important in order to confirm an underlying thrombophilic state...

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“…For venous thrombosis, varicose vein and protein S deficiency are well-documented risk factors; however, with regard to arterial thrombosis, the effects of varicose veins and protein S deficiency remain unclear[ 24 , 25 ]. In a retrospective cohort study using national health insurance data, Chang et al [ 24 ] found that varicose veins were significantly associated with peripheral arterial disease (adjusted hazard ratio, 1.76; 95%CI: 1.72-1.79).…”

Section: Discussionmentioning

confidence: 99%

“…Protein S, a cofactor of protein C, inactivates coagulation factors Va and VIIIa and inhibits thrombin generation[ 25 ]. In a retrospective family cohort study, Mahmoodi et al [ 26 ] reported that protein S deficiency increases arterial thromboembolic risk in patients below 55 years of age (adjusted hazard ratio, 4.6; 95%CI: 1.1-18.3).…”

Section: Discussionmentioning

confidence: 99%

Acute lower extremity arterial thrombosis after intraocular foreign body removal under general anesthesia: A case report and review of literature

Jeon

Hong

Lee

et al. 2021

WJCC

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BACKGROUND Surgery, which is a major risk factor for venous thrombosis, has rarely been considered a risk factor for arterial thrombosis. Recent studies have suggested that venous and arterial thromboses share common risk factors and have a bidirectional relationship. Accordingly, there is a growing interest in the risk of arterial thrombosis after surgery. We report a case of acute bilateral lower extremity arterial thromboses that developed after a prolonged surgery. CASE SUMMARY A 59-year-old man was hospitalized for intraocular foreign body removal surgery. He was a heavy-drinking smoker and had untreated hypertension and varicose veins in both legs. The operation was unexpectedly prolonged, lasting 4 h and 45 min. Immediately after emergence from general anesthesia, the patient complained of extreme pain in both legs. After the surgical drape was removed, cyanosis was evident in both feet of the patient. The pulse was not palpable, and continuous-wave Doppler signals were inaudible in the bilateral dorsalis pedis and posterior tibial arteries. Computed tomography angiography confirmed acute bilateral thrombotic occlusion of the popliteal arteries, proximal anterior tibial arteries, and tibioperoneal trunks. Arterial pulse returned in both lower limbs after 6 h of heparin initiation. The patient was discharged on postoperative day 26 without any sequelae. CONCLUSION Acute lower extremity arterial thrombosis can occur after surgery. Anesthesiologists should pay particular attention to patients with risk factors for thrombosis.

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Protein S Deficiency and Arterial Thromboembolism: A Case Report and Review of the Literature

Cited by 11 publications

References 10 publications

Influence of riboflavin and ultraviolet-light treatment on plasma proteins – protein S and alpha 2-antiplasmin – in relation to the time of administration

Influence of riboflavin and ultraviolet-light treatment on plasma proteins – protein S and alpha 2-antiplasmin – in relation to the time of administration

A Rare Thrombophilic Occurrence: Dural Venous Sinus Thrombosis in a Patient with Significant Family History of Protein S Deficiency

Acute lower extremity arterial thrombosis after intraocular foreign body removal under general anesthesia: A case report and review of literature

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