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SpeedPert SpeedPert
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6 years ago
Homozygous a-thalassemia (--/--) has what clinical outcome?
 
  a. Incompatible with life
  b. Severe anemia
  c. Mild anemia
  d. No anemia
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wrote...
6 years ago
ANS: A
When all four a genes are missing, no a globin chains are synthesized. All normal hemoglobins (A, A2, and F) require a chains. In utero, the predominant hemoglobin will be Hb Barts (g4). This is a high oxygen affinity hemoglobin and thus is not effective in reversible oxygen transport. There will also be some Hb Portland (z2g2) and traces of Hb H (b4). Hb Portland is the primary hemoglobin up until the third trimester of fetal life, so the fetus generally survives until the third trimester.
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