Faktor Risiko Timbulnya Inhibitor Faktor VIII pada Anak dengan Hemofilia A

Grace N.A. Simatupang, Endang Windiastuti, Hanifah Oswari

Sari


Latar belakang. Proses timbulnya inhibitor bersifat multifaktorial, baik genetik maupun lingkungan.
Beberapa studi telah dilakukan untuk mengetahui faktor risiko terbentuknya inhibitor, namun masih terdapat
pendapat yang kontroversial. Di Indonesia, skrining inhibitor tidak rutin dilakukan karena keterbatasan biaya
dan alat, sehingga diperlukan suatu penelitian yang dapat dijadikan acuan pemeriksaan inhibitor selektif.
Tujuan. Mengetahui prevalensi , karakteristik klinis, dan faktor risiko timbulnya inhibitor pada anak dengan
hemofilia A di Departemen IKA- RSCM.
Metode. Uji potong lintang dilakukan pada anak usia ≤18 tahun di Pusat Hemofilia Terpadu IKA-RSCM. Analisis
bivariat dilakukan dengan uji Fisher. Analisis multivariat tidak dilakukan karena tidak memenuhi syarat.
Hasil. Empatpuluh subjek penelitian, didapatkan prevalensi inhibitor 37,5% (15/40). Rentang usia subjek
10 (1,5-18) tahun, usia saat diagnosis hemofilia pertama kali ditegakkan 8 bulan, dan saat pertama kali
mendapat terapi faktor VIII pada inhibitor positif 9 bulan. Hampir seluruh subjek (39/40) mendapat terapi
konsentrat plasma, 11/15 subjek dengan inhibitor positif mendapat terapi pertama kali sebelum berusia 1
tahun, 14/15 subjek merupakan hemofilia berat, sebagian besar (12/15) mendapat manifestasi perdarahan
sendi. Suku bangsa ibu, Jawa, lebih sering ditemukan pada inhibitor positif (8/15). Tidak ditemukan hasil
yang bermakna secara statistik antara faktor risiko dengan timbulnya inhibitor.
Kesimpulan. Prevalensi inhibitor 37,5%, inhibitor positif lebih sering ditemukan pada pasien hemofilia berat
yang mendapat terapi pertama kali sebelum berusia 1 tahun. Penelitian kami tidak berhasil membuktikan faktor
risiko bermakna untuk timbulnya inhibitor pada anak dengan hemofilia A.


Kata Kunci


hemofilia A; inhibitor faktor VIII; faktor risiko

Teks Lengkap:

PDF

Referensi


The World federation of Hemophilia. Diagnosis and

management of inhibitors to factor VIII and IX: an

introductory discussion for physicians. California: The

World Federation of Hemophilia; 2004.

Van de Berg. Risk of inhibitor development in children with

hemophilia A. European Hematology. 2007;1:8-10.

Van den Berg SC. The multifactorial etiology of inhibitor

development in hemophilia: genetics and environment.

Semin Thromb Hemost 2009;35:723-34.

Lorenzo JI, Lopez A, Altisent C, Aznar JA. Incidence

of factor VIII inhibitors in severe hemophilia:

the importance of patient age. Br J Haematol

;113:600-3.

Van der Bom JG, Mauser-Bunschoten EP, Fischer K,

Van den Berg HM. Age at first treatment and immune

tolerance to factor VIII in severe hemophilia. Thromb

Haemost 2003;89:475–9.

Goudemand J, Rothschild C, Demiguel V. Influence of

the type of factor VIII concentrate on the incidence of

factor VIII inhibitors in previously untreated patients

with severe hemophilia A. Blood 2006;107:46–51.

Gouw SC, Born JG, Auerswald G, Ettinghausen CE,

Tedgard U, Berg HM. Recombinant versus plasmaderived factor VIII products and the development

of inhibitors in previously untreated patients with

severe hemophilia A: the CANAL cohort study. Blood

;109:4693-7.

Ragni MV, Ojeifo O, Feng J, Yan J, Hill KA, Sommer SS,

dkk. Risk factors for inhibitor formation in hemophilia: a

prevalent case-control study. Hemophilia 2009;15:1074-82.

Addiego J, Kasper C, Abildgaard C. Increased frequency

of inhibitors in African-American hemophilia A patients.

Blood 1994;84:239.

Aledort LM, DiMichele DM. Inhibitors occur more

frequently in African-American and Latino hemophiliacs.

Hemophilia 1998;4:68.

Xue F, Zhang L, Sui T. Factor VIII gene mutations profile

in 148 Chinese hemophilia A subjects. Eur J Hematol

;85:264-72.

Chen YC, Hu SH, Cheng SN, Chao TY. Genetic analysis

of haemophilia A in Taiwan. Hemophilia 2010;16:538-44.

Awidi A, Ramahi M, Alhattab D, Mefleh R, Dweiri M,

Bsoul N, dkk. Study of mutations in Jordanian patients

with hemophilia A: identification of five novel mutations.

Haemophilia 2010;16:136-42.

Owaidah TM, Alkhail HA, Zahrani HA, Al Musa A,

Al Saleh M, Riash MA, dkk. Molecular genotyping

of hemophilia A in Saudi Arabia: report of two novel

mutations. Blood Coagulation and Fibrinolysis

;20:415-18.

Harijadi, Djajadiman G, Akib Arwin AP. The

prevalence of factor VIII inhibitor in patients with

severe hemophilia A and its clinical characteristics.

Paediatrica Indonesiana 2005;45:7-8.

Hay CR, Palmer B, Chalmers. The incidence of factor

VIII inhibitors throughout life in severe hemophilia A

in the United Kingdom. Blood 2011;117:6367-70.

Santagostino E, Mancuso ME, Rocino A. Environmental

risk factors for inhibitor development in children with

hemophilia A: a case-control study. British J Haematol

;130:422–7.

Maclean PS, Richards M, Williams M, Collins P,

Lienser R, Keeling M, dkk. Treatment related factors

and inhibitor development in children with severe

hemophilia A. Hemophilia 2011;17:282-7.

Chalmers EA, Brown SA, Keeling D. Paediatric Working

Party of UKHCDO. Early factor VIII exposure and

subsequent inhibitor development in children with severe

hemophilia A. Hemophilia 2007;13:149–55.

Manucci PM, Gringeri I, Pevyandi F, Santagostino E.

Factor VIII products and inhibitor development: the

SIPPET study (survey of inhibitors in plasma product

exposed toddlers). Hemophilia 2007;13:65-8

Astermark J. Why do inhibitors develop? principle of and

factors influencing the risk of inhibitor development in

hemophilia. Hemophilia 2006;12:52-60.

Astermark J. Overview of Inhibitors. Semin Hematol

;43:3-7.

Gouw J, Van der Bom JG, Van den Berg HM, Zewald

RA, Van Amstel JK , Mauser-Bunschoten EP. Influence of

the type of F8 gene mutation on inhibitor development

in a single center cohort of severe hemophilia A patients.

Hemophilia 2011;17:275-81.

Miller CH, Hooper WC, Abshire TC. Factor VIII

and factor IX mutations in U.S. hemophilia patients:

Correlation with history of inhibitor and race/ethnicity.

Hemophilia 2012;18:375-82.

Han JY, Lee JN, Lee SY. Identification of factor VIII gene

mutations and carrier detection in Korean hemophilia A

patients. Hemophilia 2007;13:331-3.

Leissinger C, Cooper DL, Solem CT. Assessing the

impact of age, race, ethnicity and inhibitor status

on functional limitations of patients with severe

and moderately severe hemophilia A. Hemophilia

;17:884-9.

Gringeri A, Mantovani LG, Scalone L, Mannucci

PM. Cost of care and quality of life for patients with

hemophilia complicated by inhibitors: the COCIS study

group. Blood 2003;102: 2358–63.

Nuss R, Soucie JM, Evatt B. Changes in the occurrence

of and risk factors for hemophilia-associated intracranial

hemorrhage. Am J Hematol 2001;68:37-42.

Amelia NC, Djayadiman G, Windiastuti E, Handryastuti

S. Perdarahan intrakranial pada hemofilia: karakteristik,

tatalaksana dan luaran. Sari Pediatri 2011;13:4.




DOI: http://dx.doi.org/10.14238/sp14.5.2013.320-5

Refbacks

  • Saat ini tidak ada refbacks.


##submission.copyrightStatement##

##submission.license.cc.by-nc-sa4.footer##

Informasi Editorial:
Badan Penerbit Ikatan Dokter Anak Indonesia
Jl. Salemba I No 5, Jakarta 10430, Indonesia
Phone/Fax: +62-21-3912577
Email: editorial [at] saripediatri.org

Lisensi Creative Commons
Sari Pediatri diterbitkan oleh Badan Penerbit Ikatan Dokter Anak Indonesia
Ciptaan disebarluaskan di bawah Lisensi Creative Commons Atribusi-NonKomersial-BerbagiSerupa 4.0 Internasional.