Thromb Haemost 1976; 36(02): 441-450
DOI: 10.1055/s-0038-1648059
Original Article
Schattauer GmbH

An immunological Method for Detection of the Carrier of Hemophilia B

Matsuzo Matsuoka
1   First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
Masakazu Ito
1   First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
Kaoru Takahashi
1   First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
,
Nobuo Sakuragawa
1   First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
› Author Affiliations
Further Information

Publication History

Received 09 October 1975

Accepted 26 April 1976

Publication Date:
03 July 2018 (online)

Summary

This paper presents the results of an immunological study of hemophilia B and its carriers which used two kinds of antibodies, a heterologous antibody of high specificity and a homologous antibody developed in a patient with severe hemophilia B.

1. The factor IX related antigen for hemophilia B could be determined by a neutralization test and not by a precipitation reaction.

2. The activity of factor IX in a definite carrier of hemophilia B was significantly lower than that of factor VIII in a definite carrier of hemophilia A.

3. In hemophilia B, there were no differences between the factor IX antigen determined by either the heterologous rabbit antibodies or the homologous antibodies. And there was no discrepancy between the factor IX antigen and the factor IX activity. Therefore, we cannot use this discrepancy for the detection of a carrier of hemophilia B.

4. However, there was a discrepancy between the factor IX antigen determined by the neutralization test and the factor IX procoagulant activity in the patients of both hemophilia BM and hemophilia B+. The identical results were obtained in the cases of carriers of both hemophilia BM and hemophilia B+. These facts are very useful for the detection of carriers of hemophilia BM and hemophilia B+.

 
  • References

  • 1 Brown P. E, Hougie C, Roberts H. R. 1970; The genetic heterogeneity of hemophilia B. New England Journal of Medicine 283: 61.
  • 2 Cole E. R, Marciniac E, Seegers W. H. 1962; Procedures for the quantitative determination of autoprothrombin C. Thrombosis et Diathesis Hemorrhagica 8: 434.
  • 3 Denson K. W. E, Biggs R, Haddon M. E, Borrett R, Cobb K. 1966; “Two types of haemophilia (A+ and A)” a study of 48 cases. British Journal of Hematology 67: 163.
  • 4 Elödi S, Puskas E. 1972; Variants of haemophilia. Thrombosis et Diathesis Haemorrhagica 28: 489.
  • 5 Elödi S. 1974; Haemophilia B carrier. Lancet II 1273.
  • 6 Hanahan D. J, Kisiel W. 1973; Purification and characterization of human factor II. Biochemica et Biophysica Acta 304: 103.
  • 7 Hoyer L. W. 1972; Immunologic studies of antihemophilic factor (AHF., Factor VIII). III: Comparative binding properties of human and rabbit anti-AHF. Blood 39: 481.
  • 8 Hougie C, Towmey J. J. 1967; Haemophilia BM: a new type of factor IX deficiency. Lancet I 689.
  • 9 Ito M, Kobayashi L, Shinada S, Tsukada T, Matusoka M. 1973; Assay of factor IX related antigen by antibody neutralization test (in Japanese). Acta Haematologica Japonica 36: 379.
  • 10 Ito M, Kobayashi L, Shinada S, Tsukada T, Matsuoka M. 1974; Two cases of hemophilia B+ (North-Carolina type) in a family. (The first case in Japan) (in Japanese). Japanese Journal of Clinical Hematology 15: 629.
  • 11 Ito M, Kobayashi L, Shinada S, Matsuoka M. 1975; Studies on the detection of haemophilia carrier with special references to the immunological assay for factor VIII and factor IX (in Japanese). Japanese Journal of Clinical Hematology 16: 216.
  • 12 Matsuoka M, Tsukada T, Ito M. 1966; Examination of activated thromboplastin, standard normal plasma, prothrombin free rabbit plasma, cephaloplastin-DADE (in Japanese). Medical postgraduates 4: 1.
  • 13 Matsuoka M, Gejo F, Jimbo C, Tsukada T, Ito M. 1973; A case of hemophilia BM . (The first case in Japan) (in Japanese) Saishin-Igaku (New Medicine) 23: 380.
  • 14 Sakuragawa N, Koide T, Matsuoka M. 1975; Activation of human prothrombin by various procoagulants. Acta Medica et Biológica (Niigata) 23: 57.
  • 15 Takahashi K, Sakuragawa N, Jimbo C, Hoshiyama M, Matsuoka M, Koide T, Kotani S. 1975; Purification of factors X, IX and VII from normal human plasma (in Japanese). Japanese Journal of Clinical Hematology 16: 228.
  • 16 Zimmerman T. S, Ratnoff O. D, Littell A. S. 1971a Detection of carrier of classic hemophilia using an immunological assay for antihemophilic factor (Factor VIII). Journal of Clinical Investigation 50: 255.
  • 17 Zimmerman T. S, Ratnoff O. D, Powell A. E. 1971b Immunologic differentiation of classical hemophilia (Factor VIII deficiency) and von Willebrand disease. Journal of Clinical Investigation 50: 244.