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.