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There are two items for clarification for the hemoglobinopathies/hemolytic anemi

a lecture notes from Dr. Sarnaik:


1.
Note that although the 4 gene deletion of the alpha globin genes r
esults in almost no alpha globin synthesis, this is not complete suppression. Th
ere is still some normal hemoglobin A produced, but not enough to protect the ba
by from being still-born, unless there is treatment with intra-uterine blood tr
ansfusion. This is also the same in the case of homozygous betaO thalassemia ma
jor, the suppression is not complete and there is some normal hemoglobin A made
by the patients, but not enough to avoid severe anemia
2.
There is a typo on Page 71 of the notes. The heading for Auto-immu
ne hemolytic anemia should read Intra- vascular, Extrinsic hemolysis. In the cas
e of AIHA, the site of the hemolysis is USUALLY intravascular. In rare cases, i
f the antibody is not of a high titer, the hemolysis is milder and the hemolysis
may be also extravascular, in the reticulo-endothelium of the spleen or liver.

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