DAFTAR ISI

BAB I Pendahuluan

1.1 Latar Belakang............................................................................................ 1

BAB II Thalassemia

2.1 Definisi Thalassemia................................................................................. 3

2.2 Epidemiologi............................................................................................. 4

2.2.1 Mortalitas dan Morbiditas................................................................ 4

2.2.2 Usia................................................................................................... 5

2.3 Patofisiologi............................................................................................... 7

2.3.1 Produksi Rantai Globin.................................................................... 9

2.3.2 Patofisiologi Seluler......................................................................... 10

2.3.3 Hipotesa Malaria............................................................................... 14

2.4 Klasifikasi Thalassemia dan Presentasi Klinisnya.................................... 15

2.4.1 Thalassemia-α................................................................................... 15

a. Silent Carrier Thalassemia- α...................................................... 16

b. Trait Thalassemia-α...................................................................... 16

c. Penyakit Hb H.............................................................................. 17

d. Thalassemia-α mayor................................................................... 18

e. Thalassemia-β.............................................................................. 19

2.5 Stadium Thalassemia................................................................................. 24

2.5.1 Stadium I........................................................................................... 24

2.5.2 Stadium II......................................................................................... 25

2.5.3 Stadium III........................................................................................ 25

2.6 Pemeriksaan Penunjang..............................................................................

....................................................................................................................25

2.6.1 Darah Tepi........................................................................................ 25

2.6.2 Sumsum Tulang................................................................................ 25

2.6.3 Pemeriksaan Khusus......................................................................... 25

2.6.4 Pemeriksaan Pedigree...................................................................... 26

2.6.5 Pemeriksaan Lain............................................................................. 26

2.7 Terapi......................................................................................................... 28

2.7.1 Transfusi Darah................................................................................ 28

2.7.2 Komplikasi Transfusi Darah............................................................ 30

2.7.3 Terapi Khelasi (Pengikat Besi)........................................................ 30

2.7.4 Transplantasi Sel Stem Hematopoetik (TSSH)................................ 30

2.7.5 Terapi Bedah.................................................................................... 31

2.7.6 Diet................................................................................................... 32

2.8 Skrining....................................................................................................... 33

2.9 Prognosis..................................................................................................... 33

BAB III

Simpulan.......................................................................................................... 34

DAFTAR PUSTAKA...................................................................................... 33