Home LiteratureArticle Details
PMID: 6408124 Published · ppublish English Case Reports Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Use of human factor VIIa in the treatment of two hemophilia A patients with high-titer inhibitors.

The Journal of clinical investigation ·Vol. 71 ·No. 6 ·1983-06-00 ·Pages 1836-41

Hedner U, Kisiel W

Abstract

Two patients with hemophilia A complicated with high-titer alloantibodies have been treated by repeated infusions of microgram quantities of pure human Factor VIIa. Patient 1 was presented with a gastrocnemius muscle bleeding that involved the knee joint. Upon treatment with Factor VIIa the circumference of the muscle decreased and joint mobility increased substantially. Patient 2 was given Factor VIIa concurrent with tranexamic acid in association with the extraction of two primary molars. No significant gingival bleeding occurred after Factor VIIa and tranexamic acid treatment. Furthermore, no deleterious side effects or increase of the alloantibody level were observed in either patient throughout the Factor VIIa infusion. These results, although limited and preliminary in nature, suggest that trace quantities of Factor VIIa can act as a Factor VIII bypassing activity and restore hemostasis in these patients.

MeSH Terms
Adolescent Antigens/immunology Child Factor VII/therapeutic use Factor VIII/immunology Factor VIIa Hemophilia A/drug therapy,immunology Humans Isoantibodies/analysis Male Tooth Extraction Tranexamic Acid/therapeutic use
Chemicals
Antigens Isoantibodies Tranexamic Acid Factor VII Factor VIII Factor VIIa
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Hedner U
Kisiel W
References (21)
21 references, click to expand
  1. Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies.
    Anal Biochem. 1966 Apr;15(1):45-52 PMID: 5959431
  2. Immunologic studies in haemophilia A.
    Scand J Haematol. 1973;10(1):12-6 PMID: 4702564
  3. Tooth extractions in hemophiliacs after administration of a single dose of factor VIII or factor IX concentrate supplemented with AMCA.
    Oral Surg Oral Med Oral Pathol. 1973 Oct;36(4):482-9 PMID: 4542328
  4. Intravascular coagulation with use of human prothrombin complex concentrates.
    Ann Intern Med. 1976 Jun;84(6):683-7 PMID: 937880
  5. Proteolytic activation of protein C from bovine plasma.
    Biochemistry. 1976 Nov 2;15(22):4893-900 PMID: 990250
  6. The role of serine proteases in the blood coagulation cascade.
    Adv Enzymol Relat Areas Mol Biol. 1979;48:277-318 PMID: 367103
  7. Activated factor VII: presence in factor IX concentrates and persistence in the circulation after infusion.
    Blood. 1979 May;53(5):828-37 PMID: 435642
  8. Multiple dental extractions with a new prothrombin complex concentrate in two patients with factor VIII inhibitors.
    Thromb Res. 1979;15(3-4):359-64 PMID: 494151
  9. Studies on the thrombogenic activities in two prothrombin complex concentrates.
    Thromb Haemost. 1979 Oct 31;42(3):1022-32 PMID: 315624
  10. Activation of bovine factor XII (Hageman factor) by plasma kallikrein.
    Biochemistry. 1980 Apr 1;19(7):1322-30 PMID: 6155941
  11. Efficacy of prothrombin-complex concentrates in hemophiliacs with antibodies to factor VIII: a multicenter therapeutic trial.
    N Engl J Med. 1980 Aug 21;303(8):421-5 PMID: 6771653
  12. Prenatal diagnosis of hemophilia B by an immunoradiometric assay of factor IX.
    Blood. 1980 Sep;56(3):397-401 PMID: 7407407
  13. Anti-inhibitor Coagulant Complex (Autoplex) for treatment of factor VIII inhibitors in hemophilia.
    Blood. 1980 Dec;56(6):978-84 PMID: 6777002
  14. Hazards of prothrombin-complex concentrates in treatment of hemophilia.
    N Engl J Med. 1981 Mar 12;304(11):670-1 PMID: 7453748
  15. The effect of activated prothrombin-complex concentrate (FEIBA) on joint and muscle bleeding in patients with hemophilia A and antibodies to factor VIII. A double-blind clinical trial.
    N Engl J Med. 1981 Sep 24;305(13):717-21 PMID: 6790990
  16. Laboratory evidence of DIC under FEIBA treatment of a hemophilic patient with intracranial bleeding and high titre factor VIII inhibitor.
    Thromb Res. 1981 Apr 1-15;22(1-2):177-84 PMID: 6794176
  17. Hemophilic dog model for evaluating therapeutic effectiveness of plasma protein fractions.
    Blood. 1981 Nov;58(5):868-72 PMID: 6794675
  18. Activated factor IX complex in treatment of surgical cases of hemophilia A with inhibitors.
    Thromb Haemost. 1981 Oct;46(3):638-41 PMID: 6976016
  19. Human factor XII (Hageman factor).
    Methods Enzymol. 1981;80 Pt C:198-211 PMID: 6918768
  20. A simple, specific one-stage prothrombin assay using Russell's viper venom in cephalin suspension.
    J Lab Clin Med. 1955 Jul;46(1):89-97 PMID: 14392403
  21. The Stuart-Prower factor assay and its clinical significance.
    Thromb Diath Haemorrh. 1958 May 1;2(1-2):24-38 PMID: 13581015
Article Info
Journal
The Journal of clinical investigation
Abbr.
J Clin Invest
ISSN
0021-9738
Published
1983-06-00
Pages
1836-41
Language
English
Region
United States
NLM ID
7802877
PMCID
PMC370389
Subset
IM
Grants
NHLBI NIH HHS · HL 16919 · United States
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: [email protected]