This pathology is characterized by decreased
Hb production and red blood cell (RBC) survival, resulting
from the excess of unaffected globin chain, which form unstable
homotetramers that precipitate as inclusion bodies. -Homotetramers
in -thalassemia are more unstable than -homotetramers in
-thalassemia and therefore precipitate earlier in the RBC life span,
causing marked RBC damage and severe hemolysis associated
with ineffective erythropoiesis (IE) and extramedullary hemolysis.5