Abstract:
:We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Willebrand factor (VWF) propeptide level and a very short half-life of VWF after a test infusion with factor VIII/VWF concentrate confirmed the diagnosis AVWS. Two major surgical procedures were successfully managed using high-dose intravenous immunoglobulin. The differential diagnosis with congenital VWD and the diagnostic and therapeutic approaches of AVWS are discussed. We conclude that the diagnosis of AVWS relies primarily on clinical suspicion and a careful bleeding history. A correct diagnosis is essential for optimal perioperative management and treatment of bleeding episodes.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Eikenboom JC,Tjernberg P,Van Marion V,Heering KJdoi
10.1002/ajh.20760subject
Has Abstractpub_date
2007-01-01 00:00:00pages
55-8issue
1eissn
0361-8609issn
1096-8652journal_volume
82pub_type
杂志文章abstract::A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing mon...
journal_title:American journal of hematology
pub_type: 杂志文章
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abstract::We performed a laparoscopic splenectomy (LS) in 60 patients (age 9-83, 45 females) with idiopathic thrombocytopenic purpura (ITP) who did not achieve sustained remission on steroid therapy. Using a modified procedure, the mean duration of LS was 78 min (range 25-240 min) and surgery was associated with only 5% major a...
journal_title:American journal of hematology
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journal_title:American journal of hematology
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370306
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journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:1977-01-01 00:00:00
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journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:2006-11-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:1998-07-01 00:00:00
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journal_title:American journal of hematology
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更新日期:2012-07-01 00:00:00
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journal_title:American journal of hematology
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更新日期:1996-02-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:1999-05-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
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更新日期:2014-05-01 00:00:00
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journal_title:American journal of hematology
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journal_title:American journal of hematology
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pub_type: 杂志文章,多中心研究
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更新日期:2011-01-01 00:00:00