Clinical and prognostic implications of low or high level of von Willebrand factor in patients with Waldenstrom macroglobulinemia

被引：42

作者：

Hivert, Benedicte ^{[1
]}

Caron, Claudine ^{[2
]}

Petit, Stephanie ^{[3
]}

Charpy, Cecile ^{[4
]}

Fankam-Siaka, Corinne ^{[5
]}

Lecocq, Stephanie ^{[6
]}

Zawadzki, Christophe ^{[2
]}

Susen, Sophie ^{[2
]}

Rusu, Manuela ^{[6
]}

Duhamel, Alain ^{[7
]}

Tournilhac, Olivier ^{[8
]}

Goudemand, Jenny ^{[2
]}

Morel, Pierre ^{[1
,7
]}

机构：

[1] Hop Schaffner, Serv Hematol, Lens, France

[2] CHU Lille, Hematol Lab, F-59037 Lille, France

[3] Hop Schaffner, Serv Anat Pathol, Lens, France

[4] CHU Clermont Ferrand, Hop Estaing, Serv Anat Pathol, Clermont Ferrand, France

[5] Hop Schaffner, Hematol Lab, Lens, France

[6] Hop Schaffner, Biochim Lab, Lens, France

[7] Fac Med, Equipe Accueil 2694, Dept Biostat, Lille, France

[8] Univ Auvergne, CHU Clermont Ferrand, Hop Estaing,Equipe Accueil 7283,Serv Hematol Clin, Ctr Invest Clin 501,Inst Natl Sante & Rech Med, Clermont Ferrand, France

来源：

BLOOD | 2012年 / 120卷 / 16期

关键词：

ENDOTHELIAL-GROWTH-FACTOR; CONSENSUS PANEL RECOMMENDATIONS; 2ND INTERNATIONAL WORKSHOP; MAST-CELLS; FACTOR PROPEPTIDE; RELEASE; CYTOKINES; DISEASE;

D O I：

10.1182/blood-2011-11-388256

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Acquired von Willebrand syndrome is described in patients with Waldenstrom macroglobulinemia (WM). Assessment of ristocetin cofactor activity (VWF:RCo) and von Willebrand factor (VWF) antigen (VWF:Ag) in 72 consecutive patients with WM showed a negative relation between VWF levels < 130 U/dL and both monoclonal immunoglobulin M concentration (mIgMC) and viscosity. Ten patients with VWF: RCo < 50 U/dL (< 40 for patients with blood group O) fulfilled the acquired von Willebrand syndrome criteria. They had higher mIgMC and viscosity. Reduction in mIgMC was associated with increase in VWF levels. The low VWF:RCo/VWF: Ag ratio suggested that high viscosity might be associated with increased shear force and cleavage of multimers. Surprisingly, 43 patients (59%) presented with high VWF: Ag (> 110 U/dL). They had higher bone marrow microvessel density and vascular endothelial growth factor expression on bone marrow mast cells. Five-year survival rates of patients with VWF: Ag < 110, between 110 and 250, and more than 250 U/dL were 96%, 71%, and 44%, respectively (P < .0001). High VWF: Ag was also a significant adverse prognostic factor for survival after first-line therapy (P < .0001), independently of the international scoring system. These results support systematic assessment of VWF in patients with WM. The adverse prognostic value of high VWF levels raises issues on interactions between lymphoplasmacytic cells, mast cells, and endothelial cells in WM. (Blood. 2012; 120(16):3214-3221)

引用

页码：3214 / 3221

页数：8

共 41 条

[1] Serum concentrations of angiogenic cytokines in Waldenstrom macroglobulinaemia: the ratio of angiopoietin-1 to angiopoietin-2 and angiogenin correlate with disease severity [J].