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Abstract
Thalasemia beta adalah kondisi terjadinya mutasi dari gen beta-globin yang diwariskan dan menyebabkan berkurangnya sintesis rantai beta-globin hemoglobin. Prevalensi thalasemia beta di Indonesia mencapai 3% dari penduduk yang merupakan salah satu tertinggi dari seluruh negara di asia tenggara. Patofisiologi thalasemia beta perlu diketahui sebagai proses terjadiya gejala yang timbul dan agar dapat mengidentifikasikan pasien yang datang dengan suspek thalasemia beta. Tinjauan pustaka terkait patofisiologi berbagai gejala thalasemia beta dibuat berdasarkan temuan dari berbagai jurnal internasional yang terpercaya dan terbaru. Kondisi thalasemia beta menyebabkan adanya pemecahan dari eritrosit yang dihasilkan akibat berkurangnya rantai globin beta dan terdapat rantai globin alpha yang bebas. Kondisi pecahnya eritrosit selanjutnya menyebabkan meningkatnya kerja limpa, meningkatnya jumlah besi yang menyebabkan kelebihan besi, heme yang keluar dari eritrosit juga berubah menjadi bilirubin sehingga berlebih, dan juga terjadi hematopoiesis ekstramedular. Berbagai proses tersebut selanjutnya menyebabkan gejala-gejala pada thalasemia beta seperti splenomegali, facies cooley, anemia, ikterus, dan hepatomegali. Tiga proses yang mendasari patofisiologi pada gejala thalasemia adalah kompensasi terbentuknya sel darah merah yang tidak sempurna, berlebihnya deposit zat besi di berbagai organ, dan hematopoiesis ekstramedular. Proses tersebut dapat menjadi dasar pemahaman dari berbagai manifestasi klinik baik pada anamnesis dan pemeriksaan fisik yang muncul pada pasien thalasemia beta.
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