Acquired von Willebrand's disease: demonstration of a circulating inhibitor to the factor VIII complex in four cases

British Journal of Haematology
B M SampsonF E Preston

Abstract

We have studied four patients suffering from acquired von Willebrand's disease. All patients had a severe bleeding diathesis with recurrent life-threatening haemorrhages. Three of the patients had a monoclonal gammopathy and one of these developed multiple myeloma. In three patients tested, a plasma inhibitor to ristocetin cofactor activity was detected. In each case this was localized to the IgG fraction of plasma. In addition, VIII:C activity was found to be associated with the IgG fraction of patients' plasma and altered mobility of VIII:C was detected on Laurell immunoelectrophoresis. Furthermore, plasma from all four patients and the IgG fraction therefrom resulted in a dissociation of normal VIII:C into two components separable by gel-filtration on Sepharose 6B. Finally the circulating half-life of the three factor VIII activities was found to be markedly reduced in the patients with acquired von Willebrand's disease. We conclude that in the patients studied the coagulation defect was related to the presence of a circulating inhibitor to the factor VIII complex and that this inhibitor was associated with the IgG fraction of plasma.

References

Dec 1, 1979·The Journal of Pediatrics·P A NoronhaH S Maurer
May 4, 1978·The New England Journal of Medicine·J H JoistT S Zimmerman
Jul 1, 1978·The American Journal of Medicine·T K Rosborough, W R Swaim
Jan 1, 1978·Acta Medica Scandinavica·O Zettervall, I M Nilsson
Nov 1, 1977·British Journal of Haematology·J KouttsD Meyer
Feb 1, 1976·Scandinavian Journal of Haematology·J L WautierJ P Caen
Dec 1, 1975·British Journal of Haematology·R B Counts
Jan 1, 1987·American Journal of Hematology·A RazaH D Preisler
Jul 1, 1973·British Journal of Haematology·G I IngramJ Leslie
Jul 18, 1974·The New England Journal of Medicine·I R PeakeJ C Giddings
Oct 1, 1974·Proceedings of the Society for Experimental Biology and Medicine·C HougieR F Baugh
Jan 1, 1972·The Medical Clinics of North America·E J Bowie, C A Owen
Aug 1, 1971·British Journal of Haematology·G I IngramE J Bowie
May 1, 1980·British Journal of Haematology·L HollandD G Chalmers
Aug 1, 1980·Thrombosis Research·R G MaliaF E Preston
Nov 1, 1980·British Journal of Haematology·J H RoussiA F Goguel
Jan 1, 1955·British Journal of Haematology·R BIGGSG RICHARDS

❮ Previous
Next ❯

Citations

May 2, 1987·Lancet·R G DaltonF E Preston
Mar 24, 2005·Thyroid : Official Journal of the American Thyroid Association·Sonia SoniBetul Hatipoglu
Apr 1, 1992·British Journal of Haematology·T FaconJ Goudemand
Oct 1, 1987·Postgraduate Medical Journal·S E KinseyS J Machin
Jun 1, 1990·Postgraduate Medical Journal·N E BlesingG A McDonald
Jan 18, 2006·British Journal of Haematology·Alastair SmithUNKNOWN British Committee for Standards in Haematology
Apr 27, 2011·British Journal of Haematology·John A SnowdenUNKNOWN Haemato-oncology Task Force of British Committee for Standards in Haematology and UK Myeloma Forum
Jun 1, 1996·Baillière's Clinical Haematology·A J Cohen, C M Kessler
Nov 1, 2001·Best Practice & Research. Clinical Haematology·J J MichielsZ Berneman
May 1, 1997·Rheumatic Diseases Clinics of North America·L J Scott-Timperley, W D Haire
Apr 9, 2001·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·J J MichielsZ Berneman
Dec 3, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·I C Nitu-Whalley, C A Lee
Jul 11, 2006·American Journal of Hematology·Hiroshi Mohri
Nov 30, 2006·American Journal of Hematology·Massimo Franchini, Giuseppe Lippi
Apr 1, 1995·Haemophilia : the Official Journal of the World Federation of Hemophilia·J C EikenboomE Briët
Mar 15, 2019·Journal of Atherosclerosis and Thrombosis·Hisanori HoriuchiMasanori Matsumoto
Mar 17, 2007·Critical Reviews in Clinical Laboratory Sciences·Massimo Franchini, Giuseppe Lippi
May 4, 2011·European Journal of Haematology·Shrimati ShettyKanjaksha Ghosh
Jul 1, 2015·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Manasa Krishnegowda, Vani Rajashekaraiah
Mar 1, 1986·American Journal of Hematology·J LazarchickS A Hall
Aug 1, 1986·American Journal of Hematology·H TakahashiA Shibata
Mar 1, 1986·Scandinavian Journal of Haematology·M F López-FernándezJ Batlle
Oct 1, 1987·American Journal of Hematology·H Mohri
Feb 1, 1997·American Journal of Hematology·M R RinderH M Rinder

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.