Perbedaan Thalasemia alpha dan Thalasemia beta

Kamis, 08 Maret 2018 | Kesehatan
Perbedaan Thalasemia alpha dan Thalasemia beta -  Thalasemia adalah penyakit kelainan darah yang diakibatkan oleh faktor genetika dan menyebabkan protein yang ada di dalam sel darah merah (hemoglobin) tidak berfungsi secara normal.Hemoglobin adalah suatu zat di dalam sel darah merah yang berfungsi mengangkut zat asam dari paru-paru ke seluruh tubuh, juga memberi warna merah pada eritrosit.Thalasemia berasal dari bahasa Yunani yaitu thalasso yang berarti laut.Pertama kali ditemukan oleh seorang dokter Thomas B. Cooley tahun 1925 didaerah Laut Tengah, dijumpai pada anak-anak yang menderita anemia dengan pembesaran limfa setelah berusia satu tahun.

Perbedaan Thalasemia alpha dan Thalasemia beta
Thalasemia adalah suatu penyakit keturunan yang diakibatkan oleh kegagalan pembentukan salah satu dari empat rantai asam amino yang membentuk hemoglobin, sehingga hemoglobin tidak terbentuk sempurna


Thalasemia beta

Terdapat 2 gen yang terlibat dalam pembentukan rantai hemoglobin beta. Masing-masing orang mendapatkan 1 gen dari masing-masing orang tuanya. Thalasemia beta terdiri dari

a. Beta Thalasemia Trait.Thalasemia jenis ini memiliki satu gen normal dan satu gen yang bermutasi.Penderita mengalami anemia ringan yang ditandai dengan sel darah merah yang mengecil (mikrositer).

b. Thalasemia Intermedia.Kondisi ini kedua gen mengalami mutasi tetapi masih bisa produksi sedikit rantai beta globin. Penderita mengalami anemia yang derajatnya tergantungdari derajat mutasi gen yang terjadi.

c. Thalasemia Mayor.Kondisi ini kedua gen mengalami mutasi sehingga tidak dapat memproduksirantai beta globin. Gejala muncul pada bayi ketika berumur 3 bulan berupaanemia yang berat. Penderita thalasemia mayor tidak dapat membentukhemoglobin yang cukup sehingga hampir tidak ada oksigen yang dapatdisalurkan ke seluruh tubuh, yang lama kelamaan akan menyebabkankekurangan O2, gagal jantung kongestif, maupun kematian. Penderitathalasemia mayor memerlukan transfusi darah yang rutin dan perawatanmedis demi kelangsungan hidupnya

Thalasemia alpha

Terdapat 4 gen yang terlibat dalam pembentukan rantai hemoglobin alpha. Masing-masing orang mendapatkan 2  gen dari masing-masing orang tuanya.Thalasemia alfa terdiri dari :

a. Silent Carrier StateGangguan pada 1 rantai globin alfa. Keadaan ini tidak timbul gejala sama sekali atau sedikit kelainan berupa sel darah merah yang tampak lebih pucat.

b. Alfa Thalasemia TraitGangguan pada 2 rantai globin alpha. Penderita mengalami anemia ringan dengan sel darah merah hipokrom dan mikrositer, dapat menjadi carrier.

c. Hb H DiseaseGangguan pada 3 rantai globin alfa. Penderita dapat bervariasi mulai tidak ada gejala sama sekali, hingga anemia yang berat yang disertai denganperbesaran limpa.d. Alfa Thalassemia MayorGangguan pada 4 rantai globin alpha. Thalasemia tipe ini merupakan kondisi yang paling berbahaya pada thalassemia tipe alfa. Kondisi ini tidak terdapat rantai globin yang dibentuk sehingga tidak ada HbA atau HbF yang diproduksi. Janin yang menderita alpha thalassemia mayor pada awal kehamilan akan mengalami anemia, membengkak karena kelebihan cairan, perbesaran hati dan limpa. Janin ini biasanya mengalami keguguran atau meninggal tidak lama setelah dilahirkan.

PENGOBATAN
Penatalaksanaan untuk thalasemia mayor adalah transfusi darah secara teratur dan suplemen folat. Saat seseorang menerima transfusi darah, sebaiknya ia tidak mengonsumsi suplemen zat besi, karena hal tersebut akan meningkatkan jumlah zat besi di dalam tubuh dan merusak jantung, hati, dan sistem endokrin. Oleh karena itu, biasanya orang yang  menjalani transfusi darah akan menerima terapi chelation untuk mengeluarkan kelebihan zat besi di dalam tubuh. Transplantasi sumsum tulang dapat membantu mengobati penyakit thalasemia, terutama pada anak-anak.

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