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Acquired von Willebrand syndrome: features and management.
Am J Hematol. 2006 Aug; 81(8):616-23.AJ

Abstract

Acquired von Willebrand syndrome (AvWS) is not a well-known bleeding disorder among clinicians and is associated with various underlying diseases. The clinical manifestations are similar to congenital von Willebrand disease. Diagnosis is confirmed mainly by a decrease of ristocetin cofactor activity (vWF:RCo) and/or collagen binding activity (vWF:CBA) and by vWF multimeric analysis, usually with a selective loss of large multimers. Plasma von Willebrand factor propeptide (vWF:AgII) is a good marker of vWF synthesis. Various pathogenic mechanisms have been proposed, including development of autoantibodies to the von Willebrand factor (vWF), adsorption of vWF onto tumor cells or activated platelets, increase of vWF proteolysis, and mechanical destruction of vWF under high shear stress. Treatment of the underlying disorder may resolve AvWS. Desmopressin (DDAVP) is a first-line therapeutic option. Factor VIII/vWF concentrates and high-dose immunoglobulin infusions are reserved for patients unresponsive to DDAVP.

Authors+Show Affiliations

Division of Clinical Hematology, Department of Internal Medicine, Fujieda Municipal General Hospital, Shizuoka, Japan. mohrih@hospital.fujieda.shizuoka.jp

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

16823821

Citation

Mohri, Hiroshi. "Acquired Von Willebrand Syndrome: Features and Management." American Journal of Hematology, vol. 81, no. 8, 2006, pp. 616-23.
Mohri H. Acquired von Willebrand syndrome: features and management. Am J Hematol. 2006;81(8):616-23.
Mohri, H. (2006). Acquired von Willebrand syndrome: features and management. American Journal of Hematology, 81(8), 616-23.
Mohri H. Acquired Von Willebrand Syndrome: Features and Management. Am J Hematol. 2006;81(8):616-23. PubMed PMID: 16823821.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand syndrome: features and management. A1 - Mohri,Hiroshi, PY - 2006/7/11/pubmed PY - 2007/10/10/medline PY - 2006/7/11/entrez SP - 616 EP - 23 JF - American journal of hematology JO - Am. J. Hematol. VL - 81 IS - 8 N2 - Acquired von Willebrand syndrome (AvWS) is not a well-known bleeding disorder among clinicians and is associated with various underlying diseases. The clinical manifestations are similar to congenital von Willebrand disease. Diagnosis is confirmed mainly by a decrease of ristocetin cofactor activity (vWF:RCo) and/or collagen binding activity (vWF:CBA) and by vWF multimeric analysis, usually with a selective loss of large multimers. Plasma von Willebrand factor propeptide (vWF:AgII) is a good marker of vWF synthesis. Various pathogenic mechanisms have been proposed, including development of autoantibodies to the von Willebrand factor (vWF), adsorption of vWF onto tumor cells or activated platelets, increase of vWF proteolysis, and mechanical destruction of vWF under high shear stress. Treatment of the underlying disorder may resolve AvWS. Desmopressin (DDAVP) is a first-line therapeutic option. Factor VIII/vWF concentrates and high-dose immunoglobulin infusions are reserved for patients unresponsive to DDAVP. SN - 0361-8609 UR - https://www.unboundmedicine.com/medline/citation/16823821/Acquired_von_Willebrand_syndrome:_features_and_management_ L2 - https://doi.org/10.1002/ajh.20455 DB - PRIME DP - Unbound Medicine ER -