ABSTRACT
OBJECTIVES: The analytical performances of a new kit conceived for Hb variants separation and measurement procedures on an HPLC instrument (Tosoh HLC-723 G8) were studied. RESULTS: Between-run and within-run precision tests were satisfactory for both HbA2 and HbF measurements. HbA2 and HbF values measured using the TOSOH HLC-723 G8 were correlated to those obtained using the Bio-Rad Variant II HPLC system (r=0.974 and 0.997 respectively) and to those given by the Sebia Capillary's system (r=0.980 and 0.996 respectively). Linearity was observed for HbA2 from 2.24% to 6.56% and for HbF from 0.5 to 6%. CONCLUSION: The new analyzer Tosoh HLC-723 G8 was found to have a wide analytical range for both HbA2 and HbF. The G8 Mode beta-thal is suitable for a first-level laboratory screening for Hb analysis but also for a second-level test for the characterization of Hb variants after a first-line screening.
Subject(s)
Chromatography, High Pressure Liquid/instrumentation , Hemoglobins, Abnormal/analysis , beta-Thalassemia/blood , Automation , Calibration , Humans , Reproducibility of ResultsABSTRACT
We described the case of an infant with compound heterozygozity for a b0-thalassemic mutation and Hemoglobin (Hb) Genova, an unstable Hb variant. He has required regular transfusions as early as the second month of life and since then, behaves like a thalassemia major patient. This association leads to the most severe clinical course involving an unstable variant, reported so far.
