Immunological quantification of hemoglobins F and A2

Radial immunodiffusion techniques for hemoglobins F and A2 are described. Both techniques compare favorably with results obtained by alkali denaturation and cellulose acetate electrophoresis, respectively. Comparable results are obtained by immunoassay of hemoglobin solutions or lysed whole blood, obviating expensive equipment or washing red cells.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/22409/1/0000859.pd

The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia

A study was conducted in a sample of 140 children with sickle cell anemia to evaluate the relationship between hematological variables (%HbF, %HbA2, %Hb, and mean cell volume) and disease severity. A patient's severity status was determined by whether he/she was hospitalized, had a transfusion, and/or had a pain crisis at 2 evaluation periods; the first was based on a patient's history taken at the initial assessment visit to the Wayne State Comprehensive Sickle Cell Center, and the second was based on a 1–3 year follow-up at the center. Fetal hemoglobin was a strong predictor of a patient's hospitalization and transfusion status. A decrease in %HbF of 4.76% (one SD of %HbF) was associated with a 3.58 fold (95% confidence interval, 1.18–7.28) greater odds of being hospitalized both prior to initial assessment and on follow-up, compared to not being hospitalized at either evaluation. Similarly, a decrease in %HbF of 4.76% was associated with a 5.56 fold (95% confidence interval, 1.67–18.96) greater odds of having a transfusion both prior to initial assessment and on follow-up compared to not having a transfusion at either evaluation. Patients who were both hospitalized and transfused at initial assessment and on follow-up (n = 12) had a mean %HbF of 7.59%, while patients who were not hospitalized or transfused at either evaluation (n = 19) had a mean %HbF of 13.61%. Fetal hemoglobin was not a significant predictor of pain crises in this sample of patients. None of the other hematological variables were significant predictors of disease severity in this study. The strong relationship between %HbF and disease severity identified in this study suggests that a single %HbF measurement may be useful in predicting important aspects of the clinical course of children with sickle cell anemia.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/38246/1/1320270305_ftp.pd