Document Detail

Impaired activity of plasma von Willebrand factor-cleaving protease may predict the occurrence of hepatic veno-occlusive disease after stem cell transplantation.
MedLine Citation:
PMID:  12040478     Owner:  NLM     Status:  MEDLINE    
Hepatic veno-occlusive disease (VOD) is a life-threatening complication after stem cell transplantation (SCT), characterized by thrombus formation in hepatic venules leading to a symptom triad of hyperbilirubinemia, hepatomegaly, and ascites. Multifactorial defects in the hemostatic system may contribute to its pathogenesis, but its remains to be investigated. Unusually large VWF multimers (UL-VWFMs), produced in and released from vascular endothelial cells, are most biologically active in the interaction with platelets under a high shear stress. UL-VWFMs are cleaved and degraded into smaller VWFMs by a specific liver producing plasma protease, termed VWF-cleaving protease (VWF-CPase), which has recently been identified as a metalloprotease solely produced in liver, termed ADAMTS13. Herein, we studied the correlation between plasma VWF-CPase activity and UL-VWFMs in 21 patients who received SCT, seven patients with VOD and 14 patients without VOD. In non-VOD patients, activities (mean +/- 1s.d.) of VWF-CPase were 78 +/- 17% of the control before the conditioning regimen, 76 +/- 18% on day 0, 64 +/- 19% on day 7, 57 +/- 23% on day 14, 68 +/- 13% on day 21 and 79 +/- 19% on day 28 after SCT. The respective values in VOD patients were 32 +/- 19%, 27 +/- 15%, 18 +/- 11%, 22 +/- 18%, 26 +/- 22% and 12 +/- 4%. Thus, VWF-CPase activity was significantly reduced in VOD patients, even before the conditioning regimen, and such a difference was not found in other laboratory tests. However, despite such a clear difference, UL-VWFMs were present in plasmas of both patient groups, together with the increase of VWF antigen and ristocetin cofactor activity. These results indicate that the measurement of this enzyme activity is extremely useful in predicting the occurrence of VOD prior to a demonstration of its direct involvement in its pathogenesis.
Y-D Park; A Yoshioka; K Kawa; H Ishizashi; H Yagi; Y Yamamoto; M Matsumoto; Y Fujimura
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Bone marrow transplantation     Volume:  29     ISSN:  0268-3369     ISO Abbreviation:  Bone Marrow Transplant.     Publication Date:  2002 May 
Date Detail:
Created Date:  2002-05-31     Completed Date:  2003-07-11     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8702459     Medline TA:  Bone Marrow Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  789-94     Citation Subset:  IM    
Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
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MeSH Terms
ADAM Proteins
Case-Control Studies
Child, Preschool
Hematologic Neoplasms / complications,  therapy
Hepatic Veno-Occlusive Disease / blood,  diagnosis,  etiology*
Metalloendopeptidases / blood,  metabolism*,  standards
Predictive Value of Tests
Stem Cell Transplantation / adverse effects*
von Willebrand Factor / metabolism
Reg. No./Substance:
0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases

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