Document Detail


Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution.
MedLine Citation:
PMID:  11869938     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND AND OBJECTIVES: Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. DESIGN AND METHODS: The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. RESULTS: In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the not-MPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. INTERPRETATION AND CONCLUSIONS: AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests may be useful for screening for AVWS.
Authors:
Anal?a S?nchez-Luceros; Susana S Meschengieser; Adriana I Woods; Alicia N Blanco; Ana C Kempfer; Patricia Casais; Mar?a J Salvi?; Mar?a A Lazzari
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Haematologica     Volume:  87     ISSN:  0390-6078     ISO Abbreviation:  Haematologica     Publication Date:  2002 Mar 
Date Detail:
Created Date:  2002-02-28     Completed Date:  2002-05-22     Revised Date:  2010-03-24    
Medline Journal Info:
Nlm Unique ID:  0417435     Medline TA:  Haematologica     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  264-70     Citation Subset:  IM    
Affiliation:
Hemostasis and Thrombosis Department, Hematologic Research Institute Mariano R. Castex, National Academy of Medicine, Pacheco de Melo 3081 (C1425AUM) Buenos Aires, Argentina. sanchezluceros@hematologia.anm.edu.ar
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Child
Female
Hematologic Diseases / complications,  diagnosis
Hemorrhage / etiology
Humans
Male
Middle Aged
Myeloproliferative Disorders / complications*,  diagnosis
Retrospective Studies
Thrombosis / etiology
von Willebrand Diseases / blood,  diagnosis,  etiology*

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