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[Acquired von Willebrand syndrome].
Ned Tijdschr Geneeskd. 2003 Sep 13; 147(37):1808-11.NT

Abstract

Two patients, a man aged 69 and a woman aged 64, were diagnosed with Von-Willebrand syndrome caused by monoclonal gammopathy. The man, who was admitted for hip surgery, had a history of long episodes of epistaxis. The patient was treated with immunoglobulin and the hip operation was carried out with no complications. The woman suffered from haemorrhagic diathesis. She was advised that should she undergo an invasive procedure then treatment with a prophylactic with intravenous immunoglobulin or Von-Willebrand factor (VWF)/factor-VIII-concentrates must be administered. Acquired Von-Willebrand syndrome is a rare condition with an estimated prevalence of 0.04-0.13%. It is linked to a large number of underlying diseases such as paraproteinaemia, multiple myeloma (Kahler's disease), myeloproliferative disease, lymphoproliferative disease, auto-immune disease, solid tumours and hypothyroidism. Recognition depends on a careful case-history and identification of the underlying disease. For its diagnosis VWF antigen. VWF propeptide, activated partial thromboplastin time and factor VIII are of importance. Technically, it is difficult to show the presence of VWF antibodies as it concerns a heterogeneous group of antibodies. There are two pillars of treatment: symptomatic treatment of the bleeding tendencies using desmopressin, VWF-concentrate or intravenous gammaglobulin, and treatment of the underlying disease. The latter form of treatment can lead to acquired Von-Willebrand-syndrome disappearing altogether.

Authors+Show Affiliations

Universitair Medisch Centrum, afd. Interne Geneeskunde, F02.107, Heidelberglaan 100, 3508 GA Utrecht. d.w.delange@azu.nlNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

dut

PubMed ID

14526622

Citation

de Lange, D W., et al. "[Acquired Von Willebrand Syndrome]." Nederlands Tijdschrift Voor Geneeskunde, vol. 147, no. 37, 2003, pp. 1808-11.
de Lange DW, Fijnheer R, Wittebol S. [Acquired von Willebrand syndrome]. Ned Tijdschr Geneeskd. 2003;147(37):1808-11.
de Lange, D. W., Fijnheer, R., & Wittebol, S. (2003). [Acquired von Willebrand syndrome]. Nederlands Tijdschrift Voor Geneeskunde, 147(37), 1808-11.
de Lange DW, Fijnheer R, Wittebol S. [Acquired Von Willebrand Syndrome]. Ned Tijdschr Geneeskd. 2003 Sep 13;147(37):1808-11. PubMed PMID: 14526622.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Acquired von Willebrand syndrome]. AU - de Lange,D W, AU - Fijnheer,R, AU - Wittebol,S, PY - 2003/10/7/pubmed PY - 2003/10/31/medline PY - 2003/10/7/entrez SP - 1808 EP - 11 JF - Nederlands tijdschrift voor geneeskunde JO - Ned Tijdschr Geneeskd VL - 147 IS - 37 N2 - Two patients, a man aged 69 and a woman aged 64, were diagnosed with Von-Willebrand syndrome caused by monoclonal gammopathy. The man, who was admitted for hip surgery, had a history of long episodes of epistaxis. The patient was treated with immunoglobulin and the hip operation was carried out with no complications. The woman suffered from haemorrhagic diathesis. She was advised that should she undergo an invasive procedure then treatment with a prophylactic with intravenous immunoglobulin or Von-Willebrand factor (VWF)/factor-VIII-concentrates must be administered. Acquired Von-Willebrand syndrome is a rare condition with an estimated prevalence of 0.04-0.13%. It is linked to a large number of underlying diseases such as paraproteinaemia, multiple myeloma (Kahler's disease), myeloproliferative disease, lymphoproliferative disease, auto-immune disease, solid tumours and hypothyroidism. Recognition depends on a careful case-history and identification of the underlying disease. For its diagnosis VWF antigen. VWF propeptide, activated partial thromboplastin time and factor VIII are of importance. Technically, it is difficult to show the presence of VWF antibodies as it concerns a heterogeneous group of antibodies. There are two pillars of treatment: symptomatic treatment of the bleeding tendencies using desmopressin, VWF-concentrate or intravenous gammaglobulin, and treatment of the underlying disease. The latter form of treatment can lead to acquired Von-Willebrand-syndrome disappearing altogether. SN - 0028-2162 UR - https://www.unboundmedicine.com/medline/citation/14526622/[Acquired_von_Willebrand_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/9536 DB - PRIME DP - Unbound Medicine ER -