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Acquired von willebrand syndrome

MedGen UID:
543999
Concept ID:
C0272362
Disease or Syndrome
Synonyms: Acquired von Willebrand disease; acquired von willebrand disease; acquired von Willebrand disease; acquired von Willebrand disease (hereditary or acquired); Acquired von Willebrand syndrome; acquired von willebrand syndrome; Acquired von Willebrand's disease; AVWS; Willebrand disease, acquired
SNOMED CT: Acquired von Willebrand syndrome (234451005); Acquired von Willebrand disease (234451005); Acquired von Willebrand's disease (234451005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0020460
Orphanet: ORPHA99147

Definition

Acquired von Willebrand disease (VWD) is a rare bleeding disorder marked by the same biological anomalies as those seen in hereditary von Willebrand disease (VWD) but which occurs in association with another underlying pathology, generally in elderly patients without any personal or family history of bleeding anomalies. There are three principle pathogenic mechanisms described which are firstly the presence of autoantibodies (inhibiting or noninhibiting) that form immune complexes with the von Willebrand factor (VWF) leading to rapid clearance of VWF from the circulation. Secondly, absorption of VWF onto malignant cell clones and thirdly the increased proteolysis of high molecular weight VWF multimers under abnormal blood conditions caused by cardiovascular malformations (such as aortic valve stenosis). [from SNOMEDCT_US]

Professional guidelines

PubMed

Charlebois J, Rivard GÉ, St-Louis J
Transfus Apher Sci 2018 Dec;57(6):721-723. Epub 2018 Oct 30 doi: 10.1016/j.transci.2018.10.012. PMID: 30401518
Martin K
Curr Hematol Malig Rep 2017 Oct;12(5):389-396. doi: 10.1007/s11899-017-0400-3. PMID: 28948496
Curnow J, Pasalic L, Favaloro EJ
Semin Thromb Hemost 2016 Mar;42(2):133-46. Epub 2016 Feb 2 doi: 10.1055/s-0035-1569070. PMID: 26838696

Recent clinical studies

Etiology

Goltstein LCMJ, Rooijakkers MJP, Hoeks M, Li WWL, van Wely MH, Rodwell L, van Royen N, Drenth JPH, van Geenen EM
Eur Heart J 2023 Sep 1;44(33):3168-3177. doi: 10.1093/eurheartj/ehad340. PMID: 37555393Free PMC Article
Vandenbriele C, Arachchillage DJ, Frederiks P, Giustino G, Gorog DA, Gramegna M, Janssens S, Meyns B, Polzin A, Scandroglio M, Schrage B, Stone GW, Tavazzi G, Vanassche T, Vranckx P, Westermann D, Price S, Chieffo A
J Am Coll Cardiol 2022 May 17;79(19):1949-1962. doi: 10.1016/j.jacc.2022.02.052. PMID: 35550692
Schlagenhauf A, Kalbhenn J, Geisen U, Beyersdorf F, Zieger B
Hamostaseologie 2020 Jun;40(2):221-225. Epub 2020 May 26 doi: 10.1055/a-1150-2016. PMID: 32455462
Kreuziger LB, Massicotte MP
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):507-515. doi: 10.1182/asheducation-2018.1.507. PMID: 30504351Free PMC Article
Tzoran I, Hoffman R, Monreal M
Semin Thromb Hemost 2018 Oct;44(7):624-631. Epub 2018 Jun 19 doi: 10.1055/s-0038-1657779. PMID: 29920621

Diagnosis

Tiede A, Zieger B, Lisman T
Haemophilia 2022 May;28 Suppl 4:68-76. doi: 10.1111/hae.14548. PMID: 35521729
Sandrock-Lang K, Glonnegger H, Zieger B
Hamostaseologie 2022 Apr;42(2):117-122. Epub 2022 Apr 29 doi: 10.1055/a-1790-6156. PMID: 35488164
Tiede A, Zieger B, Lisman T
Haemophilia 2021 Feb;27 Suppl 3:5-13. Epub 2020 Jun 1 doi: 10.1111/hae.14033. PMID: 32476241
Franchini M, Mannucci PM
Haematologica 2020 Aug;105(8):2032-2037. Epub 2020 Jun 18 doi: 10.3324/haematol.2020.255117. PMID: 32554559Free PMC Article
Mital A
Adv Clin Exp Med 2016 Nov-Dec;25(6):1337-1344. doi: 10.17219/acem/64942. PMID: 28028990

Therapy

Vandenbriele C, Arachchillage DJ, Frederiks P, Giustino G, Gorog DA, Gramegna M, Janssens S, Meyns B, Polzin A, Scandroglio M, Schrage B, Stone GW, Tavazzi G, Vanassche T, Vranckx P, Westermann D, Price S, Chieffo A
J Am Coll Cardiol 2022 May 17;79(19):1949-1962. doi: 10.1016/j.jacc.2022.02.052. PMID: 35550692
Tiede A, Zieger B, Lisman T
Haemophilia 2022 May;28 Suppl 4:68-76. doi: 10.1111/hae.14548. PMID: 35521729
Rosenberg G, Siedlecki CA, Jhun CS, Weiss WJ, Manning K, Deutsch S, Pierce W
Artif Organs 2018 Dec;42(12):1119-1124. Epub 2018 Oct 28 doi: 10.1111/aor.13291. PMID: 30370640
Randi AM, Smith KE, Castaman G
Blood 2018 Jul 12;132(2):132-140. Epub 2018 Jun 4 doi: 10.1182/blood-2018-01-769018. PMID: 29866817Free PMC Article
Thomas J, Kostousov V, Teruya J
Semin Thromb Hemost 2018 Feb;44(1):20-29. Epub 2017 Sep 12 doi: 10.1055/s-0037-1606179. PMID: 28898902

Prognosis

Tiede A, Zieger B, Lisman T
Haemophilia 2022 May;28 Suppl 4:68-76. doi: 10.1111/hae.14548. PMID: 35521729
Tiede A, Zieger B, Lisman T
Haemophilia 2021 Feb;27 Suppl 3:5-13. Epub 2020 Jun 1 doi: 10.1111/hae.14033. PMID: 32476241
Horiuchi H, Doman T, Kokame K, Saiki Y, Matsumoto M
J Atheroscler Thromb 2019 Apr 1;26(4):303-314. Epub 2019 Mar 12 doi: 10.5551/jat.RV17031. PMID: 30867356Free PMC Article
Mital A
Adv Clin Exp Med 2016 Nov-Dec;25(6):1337-1344. doi: 10.17219/acem/64942. PMID: 28028990
Marongiu F, Barcellona D, Mameli A, Mariotti S
Semin Thromb Hemost 2011 Feb;37(1):11-6. doi: 10.1055/s-0030-1270066. PMID: 21305799

Clinical prediction guides

Ichinose A, Osaki T, Souri M, Favaloro EJ
Semin Thromb Hemost 2022 Nov;48(8):911-925. Epub 2022 Jul 8 doi: 10.1055/s-0042-1749088. PMID: 35803264
Tiede A, Zieger B, Lisman T
Haemophilia 2022 May;28 Suppl 4:68-76. doi: 10.1111/hae.14548. PMID: 35521729
Tiede A, Zieger B, Lisman T
Haemophilia 2021 Feb;27 Suppl 3:5-13. Epub 2020 Jun 1 doi: 10.1111/hae.14033. PMID: 32476241
Rosenberg G, Siedlecki CA, Jhun CS, Weiss WJ, Manning K, Deutsch S, Pierce W
Artif Organs 2018 Dec;42(12):1119-1124. Epub 2018 Oct 28 doi: 10.1111/aor.13291. PMID: 30370640
Nascimbene A, Neelamegham S, Frazier OH, Moake JL, Dong JF
Blood 2016 Jun 23;127(25):3133-41. Epub 2016 May 3 doi: 10.1182/blood-2015-10-636480. PMID: 27143258Free PMC Article

Recent systematic reviews

Frere C, Mazzeffi M, Maier CL, Helms J, Steiner ME, Sullenger BA, Tanaka KA, Connors JM, Levy JH
J Thromb Haemost 2024 Sep;22(9):2608-2628. Epub 2024 Jun 24 doi: 10.1016/j.jtha.2024.06.007. PMID: 38925492
Goltstein LCMJ, Rooijakkers MJP, Hoeks M, Li WWL, van Wely MH, Rodwell L, van Royen N, Drenth JPH, van Geenen EM
Eur Heart J 2023 Sep 1;44(33):3168-3177. doi: 10.1093/eurheartj/ehad340. PMID: 37555393Free PMC Article
Saha B, Wien E, Fancher N, Kahili-Heede M, Enriquez N, Velasco-Hughes A
BMJ Open Gastroenterol 2022 May;9(1) doi: 10.1136/bmjgast-2021-000866. PMID: 35534046Free PMC Article
Malfertheiner MV, Pimenta LP, Bahr VV, Millar JE, Obonyo NG, Suen JY, Pellegrino V, Fraser JF
Crit Care Resusc 2017 Oct;19(Suppl 1):45-52. PMID: 29084501

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