Thalassemias, Lepore Hemoglobins, and Hereditary Persistence of Fetal Hemoglobin HPFH

6-/3 Thalassemias, Lepore Hemoglobins, and Hereditary Persistence of Fetal Hemoglobin (HPFH) 

Hemoglobin Lepore contains abnormal 5-/3 fusion polypeptides. The N-terminal sequence in the non-j6 

HB Lepore Cooley s anemia 0 0 (and25%Hb Lepore) 75% Absence of normal J3- and 5-mRNA Normalj3-and 5-genes absent, 5j3-fusiongene present 

J3°-Thalassemia Mild disease -1- (usuallynot more than 6.5%) = or slightly + Deficiency or nonfunctional /3-mRNA -genes present 

5 -Thalassemia Mild disease 5-20% Deficiency ofj3-and 5-mRNA Deletionofj3- and 5-genes on one chromosome 

---