Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency

被引：27

作者：

Blot, E

Decaudin, D

Veyradier, A

Bardier, A

Zagame, OL

Pouillart, P

机构：

[1] Inst Curie, Dept Med Oncol, F-75231 Paris 05, France

[2] Inst Curie, Hematol Unit, F-75231 Paris, France

[3] Hop Bicetre, INSERM, U143, F-94276 Le Kremlin Bicetre, France

来源：

THROMBOSIS RESEARCH | 2002年 / 106卷 / 02期

关键词：

cancer; metastasis; thrombotic microangiopathy; Von Willebrand factor; protease;

D O I：

10.1016/S0049-3848(02)00095-6

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Cancer-related thrombotic microangiopathy (TM) is a serious complication with a short-term life-threatening prognosis. This complication shares certain similarities with thrombotic thrombocytopenic purpura. and hemolytic uremic syndrome, both characterized by circulating platelet aggregates containing ultralarge multimers of Von Willebrand factor (VWF). We report a case of cancer-related thrombotic microangiopathy secondary to disseminated metastatic cancer with undetectable serum Von Willebrand factor-cleaving protease activity and no evidence of serum inhibitory antibody. A concomitant decrease of Ca 19-9 level and hemolysis was observed during chemotherapy, in parallel with normalization of Von Willebrand factor-cleaving protease activity. The role of ultralarge multimers of Von Willebrand factor in platelet aggregation in the context of metastatic disease is discussed with respect to our findings in this case of cancer-related thrombotic microangiopathy. (C) 2002 Elsevier Science Ltd. All rights reserved.

引用

页码：127 / 130

页数：4

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