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Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies.
Acta Haematol 2009; 121(2-3):177-82AH

Abstract

Acquired von Willebrand disease (aVWD) occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative and myeloproliferative disorders, other malignancies, and cardiovascular disease. aVWD is a complex and heterogeneous defect with a multifactorial etiology and the pathophysiologic mechanisms remain unclear in many cases. Assays for anti-factor VIII (FVIII)/von Willebrand factor (VWF) activities often yield negative results although antibodies may be present in autoimmune disease and some lymphoproliferative disorders. Functional assays of VWF in patients' plasma and particularly in heart valve disease, VWF multimer analysis are important for aVWD diagnosis. In patients with normal partial thromboplastin times and normal VWF activity, the diagnosis of aVWD is based on clinical suspicion and a careful bleeding history, which should prompt the clinician to initiate further laboratory investigations. Management of bleeding in aVWD relies mainly on desmopressin, FVIII/VWF concentrates and high-dose intravenous immunoglobulin. The half-life of VWF may be very short, and in bleeding episodes high doses of FVIII/VWF concentrates at short intervals may be necessary even when high-dose intravenous immunoglobulin was applied before. Since the optimal treatment strategy has not yet been defined for aVWD of different etiology, controlled multicenter trials aiming at the development of standardized treatment protocols are urgently needed.

Authors+Show Affiliations

LaboMed Coagulation Center Berlin, Tauentzienstrasse 7 b/c, Berlin, Germany. sucker@labomed.deNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

19506364

Citation

Sucker, Christoph, et al. "Causes, Etiology and Diagnosis of Acquired Von Willebrand Disease: a Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies." Acta Haematologica, vol. 121, no. 2-3, 2009, pp. 177-82.
Sucker C, Michiels JJ, Zotz RB. Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. Acta Haematol. 2009;121(2-3):177-82.
Sucker, C., Michiels, J. J., & Zotz, R. B. (2009). Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. Acta Haematologica, 121(2-3), pp. 177-82. doi:10.1159/000214858.
Sucker C, Michiels JJ, Zotz RB. Causes, Etiology and Diagnosis of Acquired Von Willebrand Disease: a Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies. Acta Haematol. 2009;121(2-3):177-82. PubMed PMID: 19506364.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. AU - Sucker,Christoph, AU - Michiels,Jan Jacques, AU - Zotz,Rainer B, Y1 - 2009/06/08/ PY - 2009/6/10/entrez PY - 2009/6/10/pubmed PY - 2009/7/7/medline SP - 177 EP - 82 JF - Acta haematologica JO - Acta Haematol. VL - 121 IS - 2-3 N2 - Acquired von Willebrand disease (aVWD) occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative and myeloproliferative disorders, other malignancies, and cardiovascular disease. aVWD is a complex and heterogeneous defect with a multifactorial etiology and the pathophysiologic mechanisms remain unclear in many cases. Assays for anti-factor VIII (FVIII)/von Willebrand factor (VWF) activities often yield negative results although antibodies may be present in autoimmune disease and some lymphoproliferative disorders. Functional assays of VWF in patients' plasma and particularly in heart valve disease, VWF multimer analysis are important for aVWD diagnosis. In patients with normal partial thromboplastin times and normal VWF activity, the diagnosis of aVWD is based on clinical suspicion and a careful bleeding history, which should prompt the clinician to initiate further laboratory investigations. Management of bleeding in aVWD relies mainly on desmopressin, FVIII/VWF concentrates and high-dose intravenous immunoglobulin. The half-life of VWF may be very short, and in bleeding episodes high doses of FVIII/VWF concentrates at short intervals may be necessary even when high-dose intravenous immunoglobulin was applied before. Since the optimal treatment strategy has not yet been defined for aVWD of different etiology, controlled multicenter trials aiming at the development of standardized treatment protocols are urgently needed. SN - 1421-9662 UR - https://www.unboundmedicine.com/medline/citation/19506364/Causes_etiology_and_diagnosis_of_acquired_von_Willebrand_disease:_a_prospective_diagnostic_workup_to_establish_the_most_effective_therapeutic_strategies_ L2 - https://www.karger.com?DOI=10.1159/000214858 DB - PRIME DP - Unbound Medicine ER -