Performance Evaluation of a Premier Resolution HPLC System for Detecting Hemoglobin Constant Spring, Hemoglobin Paksé, and Coexisting α- and β-Thalassemia Mutations.

Abstract excerpt

BACKGROUND: Hemoglobin Constant Spring (HbCS) and Hb Paksé are clinically significant α-globin variants prevalent in Southeast Asia that cause severe non-deletional HbH disease when combined with α0-thalassemia deletions. Their inherent instability and low circulating levels (0.1%-1.0%) present analytical challenges, often yielding false negatives with conventional screening methods. This study aimed to evaluate...

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