Document Detail


Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study.
MedLine Citation:
PMID:  18208537     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Diagnosis of acquired von Willebrand syndrome (AVWS) remains challenging. Diagnostic algorithms suggest the use of factor VIII (FVIII:C), von Willebrand factor antigen (VWF:Ag), ristocetin cofactor (VWF:RCo), and collagen-binding capacity (VWF:CB), but the sensitivity of these and other laboratory tests for the diagnosis of AVWS is unknown.
OBJECTIVES: To analyze the capacity of laboratory tests, including point-of-care testing (POCT), for the identification of patients with AVWS.
PATIENTS/METHODS: Thirty-five consecutive patients were enrolled with AVWS diagnosed because of a history of recent onset of bleeding, a negative family history of von Willebrand disease, and abnormal plasma VWF multimers.
RESULTS: According to our inclusion criteria, all patients had bleeding symptoms, and the VWF high molecular weight multimers were either decreased or absent. Regarding POCT, PFA-100 was inconclusive, due to anemia or thrombocytopenia, in 29%; the sensitivity was 80% in the remaining patients. The sensitivity of VWF:Ag (23%), VWF:RCo/Ag ratio < 0.7 (26%), VWF:CB/Ag ratio < 0.7 (46%), anti-VWF antibodies (15%) and VWF propeptide/Ag ratio (22%) was too low to rule out the disease. A combination of VWF:Ag < 50 IU dL(-1), VWF:RCo/Ag ratio < 0.7 and VWF:CB/Ag ratio < 0.8 yielded a sensitivity of 86%. Patients diagnosed only because of abnormal VWF multimers showed similar clinical characteristics as other patients.
CONCLUSIONS: Early diagnosis of AVWS is difficult, due to lack of sensitivity of the tests used. A substantial number of patients present with normal or increased test results, emphasizing the importance of multimer analysis in all patients with suspected AVWS.
Authors:
A Tiede; J Priesack; S Werwitzke; K Bohlmann; B Oortwijn; P Lenting; R Eisert; A Ganser; U Budde
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-01-17
Journal Detail:
Title:  Journal of thrombosis and haemostasis : JTH     Volume:  6     ISSN:  1538-7836     ISO Abbreviation:  J. Thromb. Haemost.     Publication Date:  2008 Apr 
Date Detail:
Created Date:  2008-03-14     Completed Date:  2008-05-20     Revised Date:  2014-07-30    
Medline Journal Info:
Nlm Unique ID:  101170508     Medline TA:  J Thromb Haemost     Country:  England    
Other Details:
Languages:  eng     Pagination:  569-76     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Aged, 80 and over
Antigens / analysis*
Autoantibodies / blood*,  immunology
Autoimmune Diseases / blood,  diagnosis,  immunology
Biopolymers
Cohort Studies
Collagen / metabolism
Factor VIII / analysis*
Female
Hemorrhage / etiology
Humans
Male
Middle Aged
Molecular Weight
Platelet Function Tests / instrumentation
Platelet Glycoprotein GPIb-IX Complex / metabolism
Point-of-Care Systems
Protein Precursors / analysis*
Retrospective Studies
Sensitivity and Specificity
von Willebrand Diseases / blood,  diagnosis*,  drug therapy,  immunology
von Willebrand Factor / analysis*,  chemistry,  therapeutic use
Chemical
Reg. No./Substance:
0/Antigens; 0/Autoantibodies; 0/Biopolymers; 0/Platelet Glycoprotein GPIb-IX Complex; 0/Protein Precursors; 0/Von Willebrand antigen; 0/von Willebrand Factor; 0/von Willebrand factor propolypeptide; 9001-27-8/Factor VIII; 9007-34-5/Collagen
Comments/Corrections
Comment In:
J Thromb Haemost. 2008 Apr;6(4):565-8   [PMID:  18221357 ]

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