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Patogenesis Thalasemia
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Patogenesis Thalasemia
ThalasemiaFull description...
Author:
Gita Tanelvi
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Patogenesis Thalasemia
Mayor
NORMAL
- TALASEMIA Sintesin globin berkurang / tidak ada
Kumpulan globin α yang tidak larut mengendap di
Hb A (α2 (α22)
RBC normal
Eritoblast dalam sum-sum tulang
Eritropoiesis tidak efektif
Absorpsi Fe dalam usus ↑
Selaput eritrosit jadi mudah rusak, kelenturan ↓ & eritrosit peka thd fagositosis RES
LIEN
Pucat
Anemia Kelimpahan zat besi sistemik (hemokromatosis sekunder)
Transfusi darah
eritrosit
Kerusakan eritrosit abnormal (hemolisis)
Anoksi jaringan
Produksi EPO Hemopoeiesis ekstramedula Pengembangan sumsum / hiperaktifitas sumsum tulang Deformitas tulang
Facies talasemia dan penipisan korteks di banyak tulang
Hepatomegali
Splenomegali
Distensi Abdomen
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