Decreased Protein Z Concentrations Complicating the Hypercoagulable State of Behqet's Disease

Öztüirk, M. Akif; Özbalkan, Zeynep; Onat, A.M.; Ertenli, İhsan; Kiraz, Sedat; Aytemir, Kudret; Üreten, Kemal; Abalı, Gülcan; Çalgüineri, Meral; Kirazlı, Şerafettin; Haznedaroğlu, İbrahim C.

doi:10.1177/107602960300900312

Cited by 12 publications

(6 citation statements)

References 28 publications

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“…Decreased plasmaPZlevelsw ere also observed in patients with Behcet's disease, with amore pronounced decrease during the earlyactivephase of the disease, whereas higher levels were observedwhen disease activity declines with time (82).…”

Section: Pz Andother Arterialdiseasesmentioning

confidence: 88%

Protein Z, a protein seeking a pathology

Vasse¹

2008

Thromb Haemost

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SummaryProtein Z (PZ) is a vitamin K-dependent factor identified in human plasma in 1984 characterized by an homology with other vitamin K-dependent factors (factor VII, IX, X, protein C). In contrast to these factors, PZ does not possess any enzymatic activity but is involved as a cofactor in the down-regulation of coagulation by forming a complex with the protein Z-dependent protease inhibitor (ZPI). ZPI inhibits the activated factor X (FXa) on phospholipid surface. In mice, the disruption of PZ gene is asymptomatic, but the association with the factor V Leiden mutation leads to a quasi complete mortality during the neonatal period with microvascular thrombosis. In humans, PZ is characterized by an unusual wide distribution in plasma, and a major decrease induced by warfarin. Isolated PZ deficiency does not seem to constitute a risk for venous thrombosis, but a severe PZ deficiency could increase the risk of well recognized venous thrombotic risk factors such as factor V Leiden, G20210A mutation or hyperhomocysteinemia. Unexpectedly, a relationship between PZ deficiency and ischemic arterial diseases such as stroke, acute coronary syndromes or peripheral arterial disease was described but not confirmed by all studies. PZ deficiency could be also a risk factor for early fetal losses, and increases the arterial risk in antiphospholipid syndrome. This review analyzes the different studies so far published and discusses the various results obtained in order to understand whether or not protein Z deficiency could be considered as an arterial ischemic risk factor.

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Section: Pz Andother Arterialdiseasesmentioning

confidence: 88%

Protein Z, a protein seeking a pathology

Vasse¹

2008

Thromb Haemost

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“…[19][20][21][22][23][24] The procoagulant effect of PZ is implied in Behçet disease in which arterial and venous thrombosis is in part due to vasculitic process. 25 Protein Z levels were found to be lower in patients with preeclampsia than in healthy pregnants in a previous study. 26 We conducted this cross-sectional study to test the hypothesis that PZ would be associated with the pathogenesis and the severity of HELLP syndrome, and a cutoff level of PZ might be helpful for the prediction of disease and its severity.…”

Section: Introductionmentioning

confidence: 84%

Low Levels of Protein Z Are Associated With HELLP Syndrome and its Severity

Kaygusuz

Fıratlı-Tuğlular

Toptaş

et al. 2010

Clin Appl Thromb Hemost

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Protein Z (PZ) was found to be associated with pregnancy complications. There are no data implying an association between hemolysis (H), elevated liver enzymes (EL), and low platelet counts (LP) (HELLP) syndrome and changes in plasma levels of PZ. The aim of this study is to investigate whether HELLP syndrome is associated with plasma concentrations of PZ. Protein Z levels in 29 women with HELLP syndrome were compared with 29 healthy, nulliparous and 25 normal pregnant women. The median PZ levels in patients with HELLP syndrome were found to be significantly lower than those of pregnant women. No significant difference was found between HELLP and healthy groups. Protein Z levels correlated with platelet counts, lactate dehydrogenase (LDH), and aspartate aminotransferase (AST) levels in patients with HELLP syndrome. Median PZ level was higher in partial HELLP than in complete HELLP. We calculated 1330 ng/mL as a cutoff value for PZ level to discriminate HELLP syndrome from normal pregnancy. Low PZ levels are associated with the pathobiology of HELLP syndrome.

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“…Protein Z is another anticoagulant, a vitamin Kdependent protein acting as a co-factor in the pathway of activated factor X (FXa) inhibition. In one small case-control study, it was found to be decreased in patients with BD without vascular involvement [111].…”

Section: Anticoagulant Factorsmentioning

confidence: 96%

Behçet’s Disease as a Model of Venous Thrombosis

Regina¹,

Gasparyan²,

Orlandini³

et al. 2010

TOCMJ

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Behçet’s disease (BD) is a chronic inflammatory disease of unknown aetiology characterized by recurrent oral, genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. In this review, various factors of thrombogenesis in BD, particularly pro- and antithrombotic endothelial and non-endothelial factors, factors of coagulation, platelet activation and rheological changes are presented and discussed from positions of Virchow’s triad of venous thrombosis. Despite advances in understanding of thrombogenesis in BD, still many issues of diagnosis and targeted preventive and therapeutic measures remain unresolved. Further studies are needed to clarify the pathobiology of BD-related thrombosis and to provide the clinicians with recommendations over the utility, safety and effectiveness of the antithrombotic therapy in BD.

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Decreased Protein Z Concentrations Complicating the Hypercoagulable State of Behqet's Disease

Cited by 12 publications

References 28 publications

Protein Z, a protein seeking a pathology

Protein Z, a protein seeking a pathology

Low Levels of Protein Z Are Associated With HELLP Syndrome and its Severity

Behçet’s Disease as a Model of Venous Thrombosis

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