Pathophysiology of Thalassemia
Imbalance of globin chain synthesis results in:
ª Decreased hemoglobin
ª Microcytic hypochromic anemia
ª Unpaired globin chains precipitate
damaging cell membrane
ª Extravascular hemolysis results
from precipitated globin chains
ª Unpaired globin chains precipitate
damaging cell membrane
ª b-thalassemia
not evident until 4-6 months after birth. Why?
Hb F
containing RBCs replaced by Hb A RBCs
ª a-thalassemia
evident in fetus. Why?
Hb F
contains alpha globin chains = g4