Variabilitas Pola Perdarahan Anak Hemofilia A yang Mendapat Terapi On-demand di Rumah Sakit Cipto Mangunkusumo
Sari
Latar belakang. Gejala perdarahan pada hemofilia A bergantung pada kadar faktor VIII, namun pada kadar faktor koagulasi yang sama dapat terjadi perbedaan karakteristik dan luaran klinis.
Tujuan. Mengidentifikasi pola perdarahan, terapi dan komplikasi pada anak hemofilia A.
Metode. Penelitian kohort retrospektif pada anak ≤18 tahun di RSCM. Data diambil dari rekam medis (Januari 2014 – Juni 2016) meliputi data usia awitan perdarahan sendi, usia saat diagnosis, kekerapan perdarahan, lokasi perdarahan, penggunaan faktor VIII, dan komplikasi yang dialami.
Hasil. Terdapat 109 anak lelaki terdiri dari 2,8% hemofilia A ringan, 27,5% hemofilia A sedang, dan 69,7% hemofilia A berat. Perdarahan tersering ditemukan pada sendi (60,6%) terutama pada lutut (37,2%). Dibandingkan hemofilia A ringan dan sedang, anak hemofilia A berat menunjukkan usia awitan perdarahan sendi lebih dini (median 12,5 (4-120) bulan), kekerapan perdarahan sendi lebih sering (median 8 (1-44) kali/tahun), dan menggunakan konsentrat faktor VIII lebih banyak (median 712 (131-1913) IU/kg/tahun). Komplikasi terbanyak adalah artropati dan sinovitis kronik (46,8%) serta inhibitor faktor VIII (7,3%). Terdapat 9 dari 71 (12,6)% subjek hemofilia A berat menunjukkan karakteristik klinis lebih ringan.
Kesimpulan. Pola perdarahan pada anak hemofilia A sesuai kadar faktor VIII, tetapi pada hemofilia A berat terdapat variabilitas subjek dengan gejala klinis lebih ringan.
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DOI: http://dx.doi.org/10.14238/sp20.4.2018.221-9
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