Document Detail

Clinical study on five cases of thrombotic thrombocytopenic purpura complicating pregnancy.
MedLine Citation:
PMID:  21133861     Owner:  NLM     Status:  In-Process    
OBJECTIVE: The aim of this study was to investigate the potential role of ADAMTS13 analysis in the early recognition and management of thrombotic thrombocytopenic purpura (TTP) in pregnant women.
METHODS: Five cases of TTP were evaluated retrospectively. Clinical and laboratory findings, von Willebrand factor (vWF)-cleaving metalloprotease (ADAMTS13) activity and maternal and neonatal outcome were recorded and analysed.
RESULTS: Five cases were all nulliparous. ADAMTS13 assay was performed and the enzyme activity was less than 5% of the normal controls in three cases. Gene mutation in the 9th exon resulting in amino acid exchange 349Arg→Cys in ADAMTS13 was identified in one patient. After treatment including transfusion of fresh-frozen plasma (n = 5), packed red blood cells (n = 5), platelet transfusions (n = 2) and/or continued renal replacement therapy (CRRT) (n = 1) and plasma exchange (n = 2), three patients were alive, one died on postpartum day 6 in hospital without plasma exchange and one of familial TTP died three months after discharge.
CONCLUSION: With increasing awareness, extra-attention must be paid to patients with thrombotic microangiopathy and to measurement of ADAMTS13 activity for early diagnosis. Although severe ADAMTS13 deficiency may be helpful for TTP, it may not be sensitive enough to identify all TTP patients. Therefore, despite ADAMTS13 result positive or negative, prompt aggressive management should include early termination of pregnancy, plasma transfusion and/or plasma exchange.
Yang He; Youguo Chen; Yiming Zhao; Yuemin Zhang; Weiwen Yang
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-09-16
Journal Detail:
Title:  The Australian & New Zealand journal of obstetrics & gynaecology     Volume:  50     ISSN:  1479-828X     ISO Abbreviation:  Aust N Z J Obstet Gynaecol     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-07     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0001027     Medline TA:  Aust N Z J Obstet Gynaecol     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  519-22     Citation Subset:  IM    
Copyright Information:
© 2010 The Authors. Australian and New Zealand Journal of Obstetrics and Gynaecology © 2010 The Royal Australian and New Zealand College of Obstetricians and Gynaecologists.
Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis, Ministry of Health, Suzhou, Jiangsu, China.
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