Iranian Journal of Blood and Cancer
Iranian Journal of Blood and Cancer
Medical Sciences
http://ijbc.ir
1
admin
2008-4595
2008-4609
8
10.61186/ijbc
14
2008-4595
13
en
jalali
1391
4
1
gregorian
2012
7
1
4
4
online
1
fulltext
en
Detection of Factor VIII Inhibitors in Hemophilia A Patients
Pediatric Hematology & Oncology
Pediatric Hematology & Oncology
پژوهشي
Original Article
Background: Factor VIII administration to hemophilia A patients results in an immune response (inhibitor formation)
which significantly complicates the therapy. The present study was performed to determine the prevalence of
inhibitor development in hemophilia A patients receiving recombinant factor VIII therapy.
Materials and Methods: This was an observational descriptive study. Clotting factor inhibitor screening was
performed by activated partial thromboplastin time mixing studies using normal pool plasma collected from twenty
healthy donors. Bethesda assay for quantitation of factor VIII inhibitors was performed on samples which were
positive with screening tests.
Results: Out of 229 patients with hemophilia A enrolled in the hemophilia society of Pakistan, Lahore center, 50
patients were selected. The mean factor VIII level in these patients was 2.46 +3.14. Out of 50 patients, 29 (58%)
had severe hemophilia A (factor VIII level <1%), 17 (34%) had moderate hemophilia A (factor VIII level 1-5%) and 4
(12%) had mild hemophilia A (factor VIII level >5-30%). In this study, 12 patients (24%) were positive for inhibitors.
Most of them 9 (75%) were low responders (<5 Bethesda units) with a mean Bethesda units of 1.82+0.473, while 3
(25%) patients were high responders (>5 Bethesda units) with a mean BU of 11.33+5.85. Patients were divided into
two groups on the basis of the number of factor VIII concentrate therapies of <50 (group 1) times and >50 times
(group 2). Inhibitor positivity was high (34.5%) in group I, as compared to group II (9.5%). Bleeding episodes were
also more common in inhibitors positive patients.
Conclusion: In this study, the inhibitor development in patients with hemophilia A receiving recombinant factor VIII
concentrates therapy was 24% and the first fifty therapies were crucial for inhibitor development.
Keywords: Hemophilia A, inhibitors, Bethesda units.
Keywords: Hemophilia A, inhibitors, Bethesda units.
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Mohsin
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10031947532846001376
10031947532846001376
No
Jaffar
J
10031947532846001377
10031947532846001377
No
Hussain
Sh
10031947532846001378
10031947532846001378
Yes
Suhail
Sh
10031947532846001379
10031947532846001379
No
Ikram Ullah
M
10031947532846001380
10031947532846001380
No
Amjad
S
10031947532846001381
10031947532846001381
No