Thromb Haemost 1988; 59(03): 349-352
DOI: 10.1055/s-0038-1647493
Original Article
Schattauer GmbH Stuttgart

Bleeding Symptoms in Carriers of Hemophilia A and B

E P Mauser Bunschoten
1   The Van Creveld Clinic, Hemophilia Clinic, Medical Center “Berg en Bosch”, Bilthoven, The Netherlands
,
J C van Houwelingen
2   The Department of Mathematical Statistics, State University, Utrecht, The Netherlands
,
E J M Sjamsoedin Visser
1   The Van Creveld Clinic, Hemophilia Clinic, Medical Center “Berg en Bosch”, Bilthoven, The Netherlands
3   The University Hospital for Children and Youth “Het Wilhelmina Kinderziekenhuis”, Utrecht, The Netherlands
,
P J van Dijken
1   The Van Creveld Clinic, Hemophilia Clinic, Medical Center “Berg en Bosch”, Bilthoven, The Netherlands
3   The University Hospital for Children and Youth “Het Wilhelmina Kinderziekenhuis”, Utrecht, The Netherlands
,
A J Kok
1   The Van Creveld Clinic, Hemophilia Clinic, Medical Center “Berg en Bosch”, Bilthoven, The Netherlands
,
J J Sixma
4   The Department of Hematology State University, Utrecht, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 25 May 1986

Accepted after revision 26 October 1987

Publication Date:
29 June 2018 (online)

Summary

In order to investigate the bleeding tendency in clinically identified carriers of hemophilia, a self-administered questionnaire was held among 135 carriers of hemophilia A and B, 25 females with relatives with hemophilia and a matched group consisting of 60 females without relatives with hemophilia. Carriers of hemophilia appeared to suffer more often from bleeding than their relatives or the matched unrelated control group. A relation was seen between factor VIII :C or IX :C activity and the tendency to bleed. Obligatory carriers with normal factor VIII :C levels showed no bleeding tendency and were in this respect similar to a group of 25 females with relatives with hemophilia. This study shows that it is important to assay factor VIII: C or IX: C also in those women in whom the carrier status has already been established otherwise.

 
  • References

  • 1 Rapaport JJ, Patch MJ, Moore FJ. Antihemophilic globulin levels in carriers of hemophilia A. J Clin Invest 1961; 39: 1619-1625
  • 2 Nilsson IM, Blomback M, Ramgren O, Francken Jv. Haemophilia in Sweden. Carriers of haemophilia A and B Act Med Scand 1962; 171 (02) 223-225
  • 3 Rizza CR, Rhymes JL, Austen DEG, Kernoff PB A, Aroni SA. Detection of carriers of hemophilia a “blind” study. Brit J Haematol 1975; 30: 447-456
  • 4 Graham JB, Miller CH, Reisner HM, Elston RC, Olive JA. The phenotypic range of hemophilia A carriers. Am J Human Genetics 1976; 28: 482-488
  • 5 Chediak J, Telfer MC, Jaojaroekul T, Green D. Lower factor VIII coagulant activity in daughters of subjects with hemophilia A compared to other obligate carriers. Blood 1980; 55: 552-558
  • 6 Lyon MF. Sex chromatin and gene action in the mammilian X chromosome. Am J of Human Genetics 1962; 14: 135-148
  • 7 World Health Organisation. Methods for the detection of haemophilia carriers. A Memorandum Bulletin of the World Health Organisation 1977; 55 (06) 675-702
  • 8 Bouma BN, Starkenburg AE. Dilution of hemophilia plasma used as a reagent in the determination of anti-haemophilia factor A (Factor VIII). Haemostasis 1974; 03: 94-97
  • 9 Veltkamp JJ, Drion EF, Loeliger EA. Detection of the carrier state in hereditary coagulation disorders. I Thromb Diathes Haemorrh 1968; 19: 279-303
  • 10 Bouma BN, van der Klaauw MM, Veltkamp JJ, Starkenburg AE, van Tilburg NH, Hermans J. Evaluation of the detection rate of hemophilia carriers. Thromb Res 1975; 07: 339-350
  • 11 Fleiss J. Statistical methods for rates and proportions. 2. Wiley; New York: 1981
  • 12 Cox DR. Analysis of binary data (logistic regression analysis by means of BMDPLR computer program). Chapman and Hall; London: 1970
  • 13 Otto JC. An account of an haemorrhagic disposition existing in certain families. Am J Med 1951; 11: 557-558
  • 14 Mannucci PM, Coppola R, Lombardi R, Papa M, DeBiasi R. Direct proof of extreme lyonisation as a cause of low factor VIII levels in females. Thromb Haemostasis 1978; 39: 544-545
  • 15 Holmberg J, Nilsson IM, Henriksson P, Ostravik KI. Homozygote expression of hemophilia B in a heterozygote. Act Med Scand 1978; 204: 231-234
  • 16 Merskey C, MacFarlane RG. The female carrier of hemophilia, a clinical and laboratory study. Lancet 1951; 01: 487-490
  • 17 Wahlberg T, Blombäck M, Brodin U. Carriers and non-carriers of hemophilia A. I. Multivariate analysis of pedigree data, screening blood coagulation test and FVIII variables. Thrombosis Research 1982; 25: 401-414
  • 18 Bröcker-Vriends AHJT, Briät E, Quadt R, Dreesen JCFM, Bakker E, Claasen-Tegelaar R, Kanhai HH H, van de Kamp JJ P, Pearson PL. Genotypic assignment of haemophilia A by use of intragenic and extragenic restriction fragment length polymorphisms. Thromb Haemostasis 1987; 57: 131-136
  • 19 Gianelli F, Choo KH, Winship PR, Rizza CR, Anson DJ, Rees DJ G, Ferrari N, Brownlee GG. Characterisation and use of an intragenic polymorphic marker for detection of hemophilia B (factor IX deficiency). Lancet 1984; 01: 239-241
  • 20 Conrad ME. Diseases transmissible by blood transfusion. viral hepatitis and other infectious disorders Seminars in Haematology 1981; 18: 122
  • 21 Szmuness W, Stevens CE, Harley E, Zang EA, Oleszko WR, William DC, Sadovsky R, Morrison JM, Kellner A. Hepatitis B vaccin, demonstration of efficacy in a controlled clinical trial in a high risk population in the United States. New Engl J Med 1980; 303: 833-841