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Abnormality of von Willebrand factor

MedGen UID:
868623
Concept ID:
C4023023
Anatomical Abnormality
HPO: HP:0012146

Definition

Decreased quantity or activity of von Willebrand factor. Von Willebrand factor mediates the adhesion of platelets to the collagen exposed on endothelial cell surfaces. [from HPO]

Professional guidelines

PubMed

Castaman G, Rodeghiero F
Expert Rev Hematol 2011 Feb;4(1):95-106. doi: 10.1586/ehm.11.1. PMID: 21322782
Hambleton J
Semin Hematol 2001 Oct;38(4 Suppl 9):7-10. doi: 10.1016/s0037-1963(01)90131-3. PMID: 11685720
Castaman G, Rodeghiero F
Drugs 1995 Oct;50(4):602-14. doi: 10.2165/00003495-199550040-00003. PMID: 8536549

Recent clinical studies

Etiology

Matsumoto M, Miyakawa Y, Kokame K, Ueda Y, Wada H, Higasa S, Yagi H, Ogawa Y, Sakai K, Miyata T, Morishita E, Fujimura Y; For TTP group of Blood Coagulation Abnormalities Research Study Team, Research on Rare and Intractable diseases, Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan
Int J Hematol 2023 Nov;118(5):529-546. Epub 2023 Sep 10 doi: 10.1007/s12185-023-03657-0. PMID: 37689812Free PMC Article
Comerford C, Glavey S, Quinn J, O'Sullivan JM
J Thromb Haemost 2022 Aug;20(8):1766-1777. Epub 2022 Jun 23 doi: 10.1111/jth.15773. PMID: 35644028Free PMC Article
Warkentin TE, Kaatz S
Thromb Res 2020 Dec;196:38-51. Epub 2020 Aug 10 doi: 10.1016/j.thromres.2020.08.017. PMID: 32841919Free PMC Article
Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, Lämmle B, Matsumoto M, Pavenski K, Sadler E, Sarode R, Wu H; International Working Group for Thrombotic Thrombocytopenic Purpura
J Thromb Haemost 2017 Feb;15(2):312-322. Epub 2017 Jan 30 doi: 10.1111/jth.13571. PMID: 27868334
Montgomery RR, Marlar RA, Gill JC
Clin Haematol 1985 Jun;14(2):443-60. PMID: 3899442

Diagnosis

Matsumoto M, Miyakawa Y, Kokame K, Ueda Y, Wada H, Higasa S, Yagi H, Ogawa Y, Sakai K, Miyata T, Morishita E, Fujimura Y; For TTP group of Blood Coagulation Abnormalities Research Study Team, Research on Rare and Intractable diseases, Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan
Int J Hematol 2023 Nov;118(5):529-546. Epub 2023 Sep 10 doi: 10.1007/s12185-023-03657-0. PMID: 37689812Free PMC Article
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
Winter WE, Flax SD, Harris NS
Lab Med 2017 Nov 8;48(4):295-313. doi: 10.1093/labmed/lmx050. PMID: 29126301
Thachil J, Warkentin TE
Br J Haematol 2017 Apr;177(1):27-38. Epub 2016 Dec 16 doi: 10.1111/bjh.14482. PMID: 27982413

Therapy

Comerford C, Glavey S, Quinn J, O'Sullivan JM
J Thromb Haemost 2022 Aug;20(8):1766-1777. Epub 2022 Jun 23 doi: 10.1111/jth.15773. PMID: 35644028Free PMC Article
Tiede A, Zieger B, Lisman T
Haemophilia 2022 May;28 Suppl 4:68-76. doi: 10.1111/hae.14548. PMID: 35521729
Kumar R, Vidaurre J, Gedela S
Pediatr Neurol 2019 Sep;98:25-30. Epub 2019 May 2 doi: 10.1016/j.pediatrneurol.2019.04.019. PMID: 31201069
O'Leary JG, Greenberg CS, Patton HM, Caldwell SH
Gastroenterology 2019 Jul;157(1):34-43.e1. Epub 2019 Apr 12 doi: 10.1053/j.gastro.2019.03.070. PMID: 30986390
Tietjen GE, Collins SA
Headache 2018 Jan;58(1):173-183. Epub 2017 Feb 9 doi: 10.1111/head.13044. PMID: 28181217

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
Warkentin TE, Kaatz S
Thromb Res 2020 Dec;196:38-51. Epub 2020 Aug 10 doi: 10.1016/j.thromres.2020.08.017. PMID: 32841919Free PMC Article
Thachil J, Warkentin TE
Br J Haematol 2017 Apr;177(1):27-38. Epub 2016 Dec 16 doi: 10.1111/bjh.14482. PMID: 27982413
Tripodi A
Semin Thromb Hemost 2015 Jul;41(5):462-7. Epub 2015 Jun 16 doi: 10.1055/s-0035-1550440. PMID: 26080306

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
Chaigneau M, James PD
Hematol Oncol Clin North Am 2021 Dec;35(6):1039-1049. Epub 2021 Sep 15 doi: 10.1016/j.hoc.2021.07.001. PMID: 34535288
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
Warkentin TE, Kaatz S
Thromb Res 2020 Dec;196:38-51. Epub 2020 Aug 10 doi: 10.1016/j.thromres.2020.08.017. PMID: 32841919Free PMC Article

Recent systematic reviews

Kalot MA, Husainat N, El Alayli A, Abughanimeh O, Diab O, Tayiem S, Madoukh B, Dimassi AB, Qureini A, Ameer B, Eikenboom JCJ, Giraud N, McLintock C, McRae S, Montgomery RR, O'Donnell JS, Scappe N, Sidonio RF, Brignardello-Petersen R, Flood VH, Connell NT, James PD, Mustafa RA
Blood Adv 2022 Jan 11;6(1):62-71. doi: 10.1182/bloodadvances.2021005430. PMID: 34610118Free PMC Article
Sikora J, Karczmarska-Wódzka A, Bugieda J, Sobczak P
Int J Mol Sci 2021 Aug 10;22(16) doi: 10.3390/ijms22168605. PMID: 34445311Free PMC Article
Harmsen MJ, Wong CFC, Mijatovic V, Griffioen AW, Groenman F, Hehenkamp WJK, Huirne JAF
Hum Reprod Update 2019 Sep 11;25(5):647-671. doi: 10.1093/humupd/dmz024. PMID: 31504506Free PMC Article
Loures CMG, Duarte RCF, Silva MVF, Cicarini WB, de Souza LC, Caramelli P, Borges KBG, Carvalho MDG
Semin Thromb Hemost 2019 Jul;45(5):514-522. Epub 2019 May 16 doi: 10.1055/s-0039-1688444. PMID: 31096308
Mostmans Y, Cutolo M, Giddelo C, Decuman S, Melsens K, Declercq H, Vandecasteele E, De Keyser F, Distler O, Gutermuth J, Smith V
Autoimmun Rev 2017 Aug;16(8):774-786. Epub 2017 May 29 doi: 10.1016/j.autrev.2017.05.024. PMID: 28572048

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