Thromb Haemost 1986; 55(02): 158-161
DOI: 10.1055/s-0038-1661513
Original Article
Schattauer GmbH Stuttgart

An Inhibitor to Factor VIII: C in a Patient with Possible Combined Haemophilia A and von Willebrand’s Disease

L D Taylor
The Department of Clinical Haematology and Oncology, Royal Children’s Hospital, Melbourne, Victoria, Australia
,
F L Dean
The Department of Clinical Haematology and Oncology, Royal Children’s Hospital, Melbourne, Victoria, Australia
,
K Tiedemann
The Department of Clinical Haematology and Oncology, Royal Children’s Hospital, Melbourne, Victoria, Australia
,
H Ekert
The Department of Clinical Haematology and Oncology, Royal Children’s Hospital, Melbourne, Victoria, Australia
› Author Affiliations
Further Information

Publication History

Received 29 October 1985

Accepted 12 December 1985

Publication Date:
18 July 2018 (online)

Summary

A factor VIII inhibitor has been found in a patient with an unusual combination of factor VIII-related properties. The inhibitor is directed specifically against the clotting activity (VIII :C) of the factor VIII complex. It behaves in a similar fashion to high responding inhibitors of factor VIII seen in haemophilia A patients and it was characterised as an immunoglobulin of the IgG class. Laboratory results from the patient and his family show considerable variation of factor VUI-related properties between various individuals. Overall, the data suggests the co-existence of haemophilia A and von Willebrand’s disease in the family and the presence of both diseases in the patient.

 
  • References

  • 1 Chavin SI. Factor VIII: Structure and function in blood clotting. Am J Hematol 1984; 16: 297-306
  • 2 Zimmerman TS, Ruggeri ZM. Von Willebrand’s disease. In: Clinics in Haematology Harker LA, Zimmerman TS. (eds). Vol 12 No 1 175-200 W B Saunders Co; London: 1983
  • 3 Hoyer LW. The assessment of von Willebrand’s disease. In: The haemophilias Bloom AL. (ed) 106-121 Churchill Livingstone; Edinburgh: 1982
  • 4 Miller CH, Graham JB, Goldin LR, Elston RC. Genetics of classic von Willebrand’s disease. I. Phenotypic variation within families. Blood 1979; 54: 117-136
  • 5 Holmberg L, Nilsson IM. Haemophilia A and von Willebrand’s disease in a Swedish family. Acta Paediat Scand 1972; 61: 517-525
  • 6 Gastaldi G, Rasore-Quartino A, Galletti A, Campanella A, Barone E, Mannucci PM. Coexistence of haemophilia and von Willebrand’s disease in the same kindred. Scand J Haematol 1978; 20: 423-428
  • 7 Stormorken H, Bjomerud B, Chye NE. A new constellation: females with concomitant von Willebrand’s disease and carriership for factor VIII. Thromb Res 1982; 28: 133-136
  • 8 Tavori S, Tatarsky I. Variations in the hereditary patterns of factor VIII complex in a large kindred: co-existence of haemophilia A with von Willebrand. Am J Hematol 1984; 17: 1-9
  • 9 Dowling SV, Muntz RH, D’Souza S, Ekert H. Platelet release abnormality associated with a variant of von Willebrand’s disease. Blood 1976; 47: 265-274
  • 10 Howard MA, Salem HH, Thomas KB, Hau L, Perkin J, Coghlan M, Firkin BG. Variant von Willebrand’s disease type B - revisited. Blood 1982; 60: 1420-1428
  • 11 Taylor LD, Ekert H, Dean F. Factor VIII procoagulant antigen recovery is a dose-related response to factor VIII concentrate infusions. Haemostasis 1984; 14: 296-301
  • 12 Rizza CR, Biggs R. The treatment of patients who have factor VIII antibodies. Brit J Haematol 1973; 24: 65-82
  • 13 Bovaird JH, Ngo TT, Lenhoff HM. Optimising the o-pheny-lenediamine assay for horseradish peroxidase: Effects of phosphate and pH, substrate and enzyme concentrations, and stopping reagents. Clin Chem 1982; 28: 2423-2326
  • 14 Taylor LD, Russell RG McR, Ekert H. An immunological assay for factor VIII inhibitors. (Manuscript in preparation)
  • 15 Holmberg L, Borge L, Nilsson IM. Factor VIII :C and VIIIC:Ag response in patients with haemophilia A and von Willebrand’s disease after administration of different factor VIII concentrates or plasma. Brit J Haematol 1981; 47: 587-596
  • 16 Bennett B, Ratnoff OD, Levin J. Immunologic studies in von Willebrand’s disease. J Clin Invest 1972; 51: 2597-2601
  • 17 Weiss HJ, Sussmann II, Hoyer LW. Stabilisation of factor VIII in plasma by the von Willebrand factor. J Clin Invest 1977; 60: 390-404
  • 18 White IIG C, McMillan CW, Blatt PM, Roberts HR. Factor VIII inhibitors: a clinical overview. Am J Hematol 1982; 13: 335-342
  • 19 Mannucci PM, Mari D. Antibodies to factor VIII - von Willebrand factor in congenital and acquired von Willebrand’s disease. In: Factor VIII Inhibitors Hoyer LW. (ed) 109-122 Alan R. Liss, Inc; New York: 1984
  • 20 Miller CH, Bussel JB, Hilgartner MW. Characteristics of inhibitors in severe von Willebrand’s disease. Thromb Haemostas 1983; 50: 34 (Abstr)
  • 21 Ruggeri ZM, Ciavarella N, Mannucci PM, Molinari A, Dammacco F, Lavergne JM, Meyer D. Familial incidence of precipitating antibodies in von Willebrand’s disease: a study of four cases. J Lab Clin Med 1979; 94: 60-75