ACQUIRED VON-WILLEBRAND DISEASE-ASSOCIATED WITH MULTIPLE-MYELOMA - CHARACTERIZATION OF AN INHIBITOR TO VON-WILLEBRAND-FACTOR

被引：34

作者：

MOHRI, H ^{[1
]}

TANABE, J ^{[1
]}

OHTSUKA, M ^{[1
]}

YOSHIDA, M ^{[1
]}

MOTOMURA, S ^{[1
]}

NISHIDA, S ^{[1
]}

FUJIMURA, Y ^{[1
]}

OKUBO, T ^{[1
]}

机构：

[1] NARA MED UNIV,DEPT BLOOD TRANSFUS,NARA 634,JAPAN

来源：

BLOOD COAGULATION & FIBRINOLYSIS | 1995年 / 6卷 / 06期

关键词：

VON WILLEBRAND FACTOR; MULTIPLE MYELOMA; INHIBITOR; ACQUIRED VON WILLEBRAND DISEASE;

D O I：

10.1097/00001721-199509000-00010

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Acquired von Willebrand disease (vWD) has been described in a few patients with multiple myeloma The present study characterizes an inhibitor of von Willebrand factor (vWF) isolated from a patient with multiple myeloma (IgG-kappa). Multimeric analysis of vWF from this patient's plasma showed a reduction in multimers of all sizes. The inhibitor (IgG) detected only the vWF subunit from plasma of normal individuals. It reacted with intact vWF subunit and a 39/34kDa dispase-digested fragment of vWF (residues; Leu480/Val481-Gly718), but did not react with platelet membrane glycoproteins (GPs) or adhesive proteins. The binding of vWF to GPIb mediated by ristocetin and by botrocetin was inhibited by the patient's IgG with an IC(50)s Of 0.3 mg/ml and 0.48 mg/ml, respectively. The platelet aggregation induced by ristocetin or botrocetin was also inhibited by the IgG. These results indicate that this inhibitor may recognize the binding region of vWF to GPIb. Therefore, the antibody to vWF appears to represent the likely pathophysiological mechanism responsible for the acquired vWD in this patient.

引用

页码：561 / 566

页数：6

共 27 条

[1] CROSS-LINKING OF A MONOMERIC 39/34-KDA DISPASE FRAGMENT OF VONWILLEBRAND-FACTOR (LEU-480/VAL-481-GLY-718) TO THE N-TERMINAL REGION OF THE ALPHA-CHAIN OF MEMBRANE GLYCOPROTEIN-IB ON INTACT PLATELETS WITH BIS(SULFOSUCCINIMIDYL) SUBERATE [J].