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Abnormal factor VIII activity

MedGen UID:
1387304
•Concept ID:
C4476889
•
Finding
HPO: HP:0030976

Definition

A deviation from the normal activity of coagulation factor VIII. Factor VIII (fVIII) is a cofactor in the intrinsic clotting cascade that is activated to fVIIIa in the presence of minute quantities of thrombin. fVIIIa acts as a receptor, for factors IXa and X. [from HPO]

Professional guidelines

PubMed

Management of coagulation factor VIII (FVIII) inhibitors.
Lassila R
Thromb Res 2019 Sep;181 Suppl 1:S60-S61. doi: 10.1016/S0049-3848(19)30369-X. PMID: 31477231
A new era of treatment for patients with haemophilia A?
Klamroth R
Hamostaseologie 2017 Aug 8;37(3):216-218. Epub 2016 Nov 25 doi: 10.5482/HAMO-16-07-0028. PMID: 27885373
Screening and diagnosis of coagulation disorders.
Lusher JM
Am J Obstet Gynecol 1996 Sep;175(3 Pt 2):778-83. doi: 10.1016/s0002-9378(96)80084-6. PMID: 8828561

Recent clinical studies

Etiology

The role of VWF/FVIII in thrombosis and cancer progression in multiple myeloma and other hematological malignancies.
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
Update on the Clot Waveform Analysis.
Wada H, Matsumoto T, Ohishi K, Shiraki K, Shimaoka M
Clin Appl Thromb Hemost 2020 Jan-Dec;26:1076029620912027. doi: 10.1177/1076029620912027. PMID: 32862666Free PMC Article
How I treat von Willebrand disease.
Castaman G
Thromb Res 2020 Dec;196:618-625. Epub 2020 Aug 3 doi: 10.1016/j.thromres.2020.07.051. PMID: 32819724
The von Willebrand factor.
Rodeghiero F, Castaman G
Ric Clin Lab 1990 Apr-Jun;20(2):143-53. doi: 10.1007/BF02877561. PMID: 2196663
Juvenile dermatomyositis.
Pachman LM
Pediatr Clin North Am 1986 Oct;33(5):1097-117. doi: 10.1016/s0031-3955(16)36110-7. PMID: 3532004

Diagnosis

How I treat von Willebrand disease.
Castaman G
Thromb Res 2020 Dec;196:618-625. Epub 2020 Aug 3 doi: 10.1016/j.thromres.2020.07.051. PMID: 32819724
Management of coagulation factor VIII (FVIII) inhibitors.
Lassila R
Thromb Res 2019 Sep;181 Suppl 1:S60-S61. doi: 10.1016/S0049-3848(19)30369-X. PMID: 31477231
A new era of treatment for patients with haemophilia A?
Klamroth R
Hamostaseologie 2017 Aug 8;37(3):216-218. Epub 2016 Nov 25 doi: 10.5482/HAMO-16-07-0028. PMID: 27885373
Clinical and laboratory diagnosis of VWD.
Federici AB
Hematology Am Soc Hematol Educ Program 2014 Dec 5;2014(1):524-30. Epub 2014 Nov 18 doi: 10.1182/asheducation-2014.1.524. PMID: 25696905
Juvenile dermatomyositis.
Pachman LM
Pediatr Clin North Am 1986 Oct;33(5):1097-117. doi: 10.1016/s0031-3955(16)36110-7. PMID: 3532004

Therapy

Current and future therapies for haemophilia-Beyond factor replacement therapies.
Nogami K, Shima M
Br J Haematol 2023 Jan;200(1):23-34. Epub 2022 Jul 23 doi: 10.1111/bjh.18379. PMID: 35869698
The role of VWF/FVIII in thrombosis and cancer progression in multiple myeloma and other hematological malignancies.
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
How I treat von Willebrand disease.
Castaman G
Thromb Res 2020 Dec;196:618-625. Epub 2020 Aug 3 doi: 10.1016/j.thromres.2020.07.051. PMID: 32819724
A new era of treatment for patients with haemophilia A?
Klamroth R
Hamostaseologie 2017 Aug 8;37(3):216-218. Epub 2016 Nov 25 doi: 10.5482/HAMO-16-07-0028. PMID: 27885373
Antihemophilic factor (factor VIII).
Ratnoff OD
Ann Intern Med 1978 Mar;88(3):403-9. doi: 10.7326/0003-4819-88-3-403. PMID: 343683

Prognosis

Management of coagulation factor VIII (FVIII) inhibitors.
Lassila R
Thromb Res 2019 Sep;181 Suppl 1:S60-S61. doi: 10.1016/S0049-3848(19)30369-X. PMID: 31477231
Inflammatory bowel disease: epidemiology, pathology and risk factors for hypercoagulability.
Owczarek D, Cibor D, Głowacki MK, Rodacki T, Mach T
World J Gastroenterol 2014 Jan 7;20(1):53-63. doi: 10.3748/wjg.v20.i1.53. PMID: 24415858Free PMC Article
Procoagulant activity in hemostasis and thrombosis: Virchow's triad revisited.
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Hemorrhagic and thrombotic disorders due to factor V deficiencies and abnormalities: an updated classification.
Girolami A, Simioni P, Scarano L, Girolami B, Marchiori A
Blood Rev 1998 Mar;12(1):45-51. doi: 10.1016/s0268-960x(98)90029-7. PMID: 9597197
The von Willebrand factor.
Rodeghiero F, Castaman G
Ric Clin Lab 1990 Apr-Jun;20(2):143-53. doi: 10.1007/BF02877561. PMID: 2196663

Clinical prediction guides

Current and future therapies for haemophilia-Beyond factor replacement therapies.
Nogami K, Shima M
Br J Haematol 2023 Jan;200(1):23-34. Epub 2022 Jul 23 doi: 10.1111/bjh.18379. PMID: 35869698
Clinical and laboratory diagnosis of VWD.
Federici AB
Hematology Am Soc Hematol Educ Program 2014 Dec 5;2014(1):524-30. Epub 2014 Nov 18 doi: 10.1182/asheducation-2014.1.524. PMID: 25696905
Inflammatory bowel disease: epidemiology, pathology and risk factors for hypercoagulability.
Owczarek D, Cibor D, Głowacki MK, Rodacki T, Mach T
World J Gastroenterol 2014 Jan 7;20(1):53-63. doi: 10.3748/wjg.v20.i1.53. PMID: 24415858Free PMC Article
Procoagulant activity in hemostasis and thrombosis: Virchow's triad revisited.
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
The von Willebrand factor.
Rodeghiero F, Castaman G
Ric Clin Lab 1990 Apr-Jun;20(2):143-53. doi: 10.1007/BF02877561. PMID: 2196663

Recent systematic reviews

Anti-epidermal growth factor receptor therapy for glioblastoma in adults.
Lee A, Arasaratnam M, Chan DLH, Khasraw M, Howell VM, Wheeler H
Cochrane Database Syst Rev 2020 May 12;5(5):CD013238. doi: 10.1002/14651858.CD013238.pub2. PMID: 32395825Free PMC Article
Role of angiogenesis in adenomyosis-associated abnormal uterine bleeding and subfertility: a systematic review.
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
Hemostatic Abnormalities in Dementia: A Systematic Review and Meta-Analysis.
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

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