Contribution of natural anticoagulant and fibrinolytic factors in modulating the clinical severity of haemophilia patients

被引：79

作者：

Shetty, Shrimati ^{[1
]}

Vora, Sonal ^{[1
]}

Kulkarni, Bipin ^{[1
]}

Mota, Leenam ^{[1
]}

Vijapurkar, Manasi ^{[1
]}

Quadros, Leera ^{[1
]}

Ghosh, Kanjaksha ^{[1
]}

机构：

[1] King Edward Mem Hosp, ICMR, Inst Immunoharmatol, Bombay 400012, Maharashtra, India

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2007年 / 138卷 / 04期

关键词：

haemophilia; milder manifestation; prothrombotic markers; fibrinolytic components;

D O I：

10.1111/j.1365-2141.2007.06693.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The role of natural anticoagulants, fibrinolytic cascade factors and common prothrombotic gene polymorphisms in modulating disease severity were studied in 35 'clinically mild' and 37 'clinically severe' haemophilia patients with severe factor VIII or IX deficiency (< 0.01 IU/ml). Strong association of deficiencies of proteins C and S, antithrombin III, tissue factor pathway inhibitor and tissue plasminogen activator, together with factor V Leiden and endothelial protein C receptor 23 bp insertion polymorphisms were observed in the 'clinically milder' group as compared with the 'clinically severe' group. These results indicate a synergistic modulation of bleeding tendency in haemophilia patients by factors in the anticoagulant and fibrinolytic systems.

引用

页码：541 / 544

页数：4

共 11 条

[1]

ARBINI AA, 1995, THROMB HAEMOSTASIS, V74, P1255

[2] Coinheritance of Factor V (FV) Leiden enhances thrombin formation and is associated with a mild bleeding phenotype in patients homozygous for the FVII 9726+5G>A (FVII Lazio) mutation [J].

Castoldi, E ;

Govers-Riemslag, JWP ;

Pinotti, M ;

Bindini, D ;

Tans, G ;

Berettini, M ;

Mazzucconi, MG ;

Bernardi, F ;

Rosing, J .

BLOOD, 2003, 102 (12) :4014-4020

[3]

Ettingshausen CE, 2001, THROMB HAEMOSTASIS, V85, P218

[4] Milder clinical presentation of haemophilia A with severe deficiency of factor VIII as measured by one-stage assay [J].

Ghosh, K ;

Shetty, S ;

Mohanty, D .

HAEMOPHILIA, 2001, 7 (01) :9-12

[5] Venous thromboembolism in young patients from Western India: A study [J].

Ghosh, K ;

Shetty, S ;

Madkaikar, M ;

Pawar, A ;

Nair, S ;

Khare, A ;

Pathare, A ;

Jijina, F ;

Mohanty, D .

CLINICAL AND APPLIED THROMBOSIS-HEMOSTASIS, 2001, 7 (02) :158-165

[6]

Lindqvist PG, 1998, THROMB HAEMOSTASIS, V79, P69

[7] Moderation of hemophilia A phenotype by the factor V R506Q mutation [J].

Nichols, WC ;

Amano, K ;

Cacheris, PM ;

Figueiredo, MS ;

Michaelides, K ;

Schwaab, R ;

Hoyer, L ;

Kaufman, RJ ;

Ginsburg, D .

BLOOD, 1996, 88 (04) :1183-1187

[8]

Tizzano EF, 2002, HAEMATOLOGICA, V87, P279

[9] An in vitro analysis of the combination of hemophilia a and factor V-LEIDEN [J].

vantVeer, C ;

Golden, NJ ;

Kalafatis, M ;

Simioni, P ;

Bertina, RM ;

Mann, KG .

BLOOD, 1997, 90 (08) :3067-3072

[10] Mild bleeding diathesis in a boy with combined severe haemophilia B (C10400→T) and heterozygous factor V Leiden [J].

Vianello, F ;

Belvini, D ;

Dal Bello, F ;

Tagariello, G ;

Zanon, E ;

Lombardi, AM ;

Zerbinati, P ;

Girolami, A .

HAEMOPHILIA, 2001, 7 (05) :511-514

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