The prevalence of factor VIII inhibitor in patients with severe hemophilia-A and its clinical characteristics
Keywords:
severe hemophilia-A, factor VIII inhibitor, prevalence
Abstract
Background Hemophilia is a hereditary blood-clotting disorderdue to factor VIII deficiency. Up to this date, the administration of
factor VIII in preventing and managing bleeding has been the main
treatment. One of the complications, which may occur due to re-
peated administrations of factor VIII, is the formation of factor VIII
antibody (factor VIII inhibitor).
Objective To find out the prevalence of severe hemophilia-A with
factor VIII inhibitor and its clinical characteristics.
Methods A cross-sectional descriptive study was performed on
children with severe hemophilia-A at the National Hemophilia Care
Centre, Cipto Mangunkusumo Hospital, Jakarta, Indonesia, in June-
August 2004.
Results Out of 45 children studied, 16 had factor VIII inhibitor with
average inhibitor titre of 1.15 Bethesda units (BU) (range 0.15-15
BU). Most of them (12 patients) had inhibitor titre <5 BU. Chronic
arthropathy was found in 17 out of 45 (37%) children with severe
hemophilia-A, consisting of nine patients from positive inhibitor
group and 8 patients from negative inhibitor group. Thirty-nine
patients (86%) used an on-demand treatment pattern, among whom
15 had positive inhibitor. Among patients receiving prophylactic
treatment pattern, only one had positive inhibitor. There were 39
patients (86%) treated using cryoprecipitate, among whom factor
VIII inhibitor was found in 12, while among those treated with fac-
tor VIII concentrate, the inhibitor was positive in 4/6. The average
amount of factor VIII transfused in positive and negative factor VIII
inhibitor groups was similar.
Conclusion The prevalence of factor VIII inhibitor in severe he-
mophilia-A patients was 35%. Chronic arthropathy occurred more
often in patients with positive factor VIII inhibitor. Factor VIII inhibi-
tor was found more frequently in patients with an on-demand treat-
ment pattern and in those using factor VIII concentrate
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botic diseases. In: Behrman RE, Kliegman RM, Jenson
HB, editors. Nelson textbook of pediatrics. 16th ed.
Philadelphia: W.B. Saunders Company; 2000. p. 1509-
13.
2. Rodgers GM, Greenberg CS. Clinical evaluation of the
bleeding patient. In: Lee GR, Foerster J, Lukens J,
Paraskevas F, Greer JP, Rodgers GM, editors. Wintrobe’s
clinical hematology. 10th ed. Baltimore: Lippincott
Williams & Wilkins; 1999. p. 1686-92.
3. Furrie B, Limetans SA, Rosen Field CG. A practical
guide to evaluations and treatment of hemophilia.
Blood 1994;84:3-9.
4. Jones P. Living with hemophilia. 4th ed. New York:
Oxford University Press; 1995. p. 205-14.
5. Brettler DB. Inhibitors of factor VIII and IX. Hemo-
philia 1995;1:35-9.
6. Roberts HR. The pathophysiology, pathogenesis, and
management of inhibitors in patients with hemophilia-
A and B. Proceedings of the 9th International Con-
gress of Society on Haematology Asian-Pasific division;
1999 Oct 24-28; Bangkok, Thailand.
7. Vermylen J. How do some hemophiliacs develops in-
hibitors? Hemophilia 1998;4:538-42.
8. Tuddenham GD, McVey JH. The genetic basis of in-
hibitor development in hemophilia A. Hemophilia
1998;4:543-5.
9. Gilles JG. Pathogenesis of inhibitors. Hemophilia
2000;6:13-28.
10. Lorenzo JI, Altisent C, Aznar JA. Incidence of factor
VIII inhibitors in severe hemophilia: the importance
of patient age. Br J Haematol 2001;113:600-3.
11. Giangrande PLF. Inhibitory antibodies. Blood Coagu-
lation Fibrinolysis 1996;7:134-8.
12. Goudemand J. Treatment of patients with inhibitors:
cost issues. Hemophilia 1995;5:397-401.
13. Gilles JGG, Jacqueemin MG, Saint-Remy JMR. Factor
VIII inhibitors. Thrombosis and Haemostasis 1997; 78:
641-6.
14. DiMichele DM. Inhibitors: resolving diagnostic and
therapeutic dilemmas. Hemophilia 2002;8:280-7.
15. Brettler DB. Inhibitors in congenital hemophilia. In:
Lee CA, editor. Bailliere’s clinical haematology. Lon-
don: WB Saunders; 1996. p. 319-29.
16. El Alfy MS, Tantawy AA, Ahmed MH, Abdin IA. Fre-
quency of inhibitor development in severe hemophilia
A children treated with cryoprecipitate and low-dose
immune. Hemophilia 2000;6:635-8.
17. Kavakli K, Gringeri A, Bader R, Nisli G, Polat A,
Aydinok Y. Inhibitor development and substitution
treatment in a developing country: Turkey. Hemophilia
1998;4:104-8.
18. Lusher JM. Considerations for current and future man-
agement of hemophilia and its complications. Hemo-
philia 1995;1:2-10.
19. Giles AR, Rivard GE, Teitel J, Walker I. Suggestion for
the management of factor VIII inhibitors. World fed-
eration of hemophilia. Hemophilia 1997;10:1-16.
Published
2016-10-10
How to Cite
1.
Harijadi H, Gatot D, Akib A. The prevalence of factor VIII inhibitor in patients with severe hemophilia-A and its clinical characteristics. PI [Internet]. 10Oct.2016 [cited 27Apr.2024];45(4):177-1. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/832
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Received 2016-10-08
Accepted 2016-10-08
Published 2016-10-10
Accepted 2016-10-08
Published 2016-10-10
