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Factor VIII Inactivation

MedGen UID:
1389268
•Concept ID:
C4329969
•
Disease or Syndrome

Definition

Presence of inactivating antibodies to factor VIII in the blood. [from NCI]

Professional guidelines

PubMed

The relevance of factor VIII inactivation characteristics in the treatment of patients with antibodies directed against factor VIII.
Kernoff PB
Br J Haematol 1972 Jun;22(6):735-42. doi: 10.1111/j.1365-2141.1972.tb05718.x. PMID: 5064501

Recent clinical studies

Etiology

Increased factor VIII coagulant activity levels in male carriers of the factor V R2 polymorphism.
Martinelli N, Girelli D, Ferraresi P, Olivieri O, Lunghi B, Manzato F, Corrocher R, Bernardi F
Blood Coagul Fibrinolysis 2007 Mar;18(2):125-9. doi: 10.1097/MBC.0b013e328011c06d. PMID: 17287628
Classification of the kinetics of factor VIII inhibitors in haemophilia A: plasma dilution studies are more discriminatory than time-course studies.
Ling M, Duncan EM, Rodgers SE, Somogyi AA, Crabb GA, Street AM, Lloyd JV
Br J Haematol 2001 Sep;114(4):861-7. doi: 10.1046/j.1365-2141.2001.03067.x. PMID: 11564075
Factor V antigen levels and venous thrombosis: risk profile, interaction with factor V leiden, and relation with factor VIII antigen levels.
Kamphuisen PW, Rosendaal FR, Eikenboom JC, Bos R, Bertina RM
Arterioscler Thromb Vasc Biol 2000 May;20(5):1382-6. doi: 10.1161/01.atv.20.5.1382. PMID: 10807757
Acquired protein C dysfunction but not decreased activity of thrombomodulin is a possible marker of thrombophilia in patients with lupus anticoagulant.
Pötzsch B, Kawamura H, Preissner KT, Schmidt M, Seelig C, Müller-Berghaus G
J Lab Clin Med 1995 Jan;125(1):56-65. PMID: 7822946

Diagnosis

Increased factor VIII coagulant activity levels in male carriers of the factor V R2 polymorphism.
Martinelli N, Girelli D, Ferraresi P, Olivieri O, Lunghi B, Manzato F, Corrocher R, Bernardi F
Blood Coagul Fibrinolysis 2007 Mar;18(2):125-9. doi: 10.1097/MBC.0b013e328011c06d. PMID: 17287628

Therapy

Activation, activity and inactivation of factor VIII in factor VIII products.
Orfeo T, Elsman R, Gissel M, Mann KG, Butenas S
Haemophilia 2016 May;22(3):462-73. Epub 2016 Jan 29 doi: 10.1111/hae.12872. PMID: 26822998
The relevance of factor VIII inactivation characteristics in the treatment of patients with antibodies directed against factor VIII.
Kernoff PB
Br J Haematol 1972 Jun;22(6):735-42. doi: 10.1111/j.1365-2141.1972.tb05718.x. PMID: 5064501

Prognosis

Classification of the kinetics of factor VIII inhibitors in haemophilia A: plasma dilution studies are more discriminatory than time-course studies.
Ling M, Duncan EM, Rodgers SE, Somogyi AA, Crabb GA, Street AM, Lloyd JV
Br J Haematol 2001 Sep;114(4):861-7. doi: 10.1046/j.1365-2141.2001.03067.x. PMID: 11564075

Clinical prediction guides

Factor V enhances the cofactor function of protein S in the APC-mediated inactivation of factor VIII: influence of the factor VR506Q mutation.
Váradi K, Rosing J, Tans G, Pabinger I, Keil B, Schwarz HP
Thromb Haemost 1996 Aug;76(2):208-14. PMID: 8865533
The activities of recombinant gamma-carboxyglutamic-acid-deficient mutants of activated human protein C toward human coagulation factor Va and factor VIII in purified systems and in plasma.
Jhingan A, Zhang L, Christiansen WT, Castellino FJ
Biochemistry 1994 Feb 22;33(7):1869-75. doi: 10.1021/bi00173a033. PMID: 8110790
Inactivation of factor VIII by activated protein C and protein S.
Walker FJ, Chavin SI, Fay PJ
Arch Biochem Biophys 1987 Jan;252(1):322-8. doi: 10.1016/0003-9861(87)90037-3. PMID: 2949697

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