Abstract
α0-Thalassemia is an inherited hematological disorder caused by the deletion of α-globin genes. The Southeast Asian deletion (--SEA) is the most common type of α0-thalassemia observed in Southeast Asian countries. Regarding WHO health policy, an effective α0-thalassemia screening strategy is needed to control new severe α-thalassemia cases. In this study, a monoclonal antibody panel was used to develop immunochromatographic (IC) strip tests for detecting the Hb Bart’s and ζ-globin chain. Among 195 samples, all α0-thalassemia traits (78 α0-thalassemia (--SEA) and 4 α0-thalassemia (--THAI)) had low MCV or MCH values. The sensitivity, specificity, PPV and NPV of the IC strip tests for ζ-globin and Hb Bart’s for screening α0-thalassemia (--SEA) within the low MCV or MCH samples were 100%, 65.2%, 90.7%, 100% and 96.2%, 47.8%, 86.6%, 78.6%, respectively. All 4 α0-thalassemia (--THAI) traits were negative for ζ-globin chains but positive for Hb Bart’s using the IC strip tests. These results led to a α0-thalassemia screening being proposed in which blood samples are first evaluated by MCV, MCH and Hb typing. Samples with high MCV and MCH values are excluded for the presence of the α0-thalassemia gene. Samples with low MCV or MCH values are assayed using the developed IC strip tests, where only samples testing positive are further assayed for α0-thalassemia by PCR. Patients with Hb H, EA Bart’s or EF Bart’s diseases do not need to use this IC strip assay. Thus, in this study, a simple and cost effective α0-thalassemia point of care test was developed.
Highlights
Introduction αThalassemia is a genetic disorder caused by a defect in the α-globin gene [1, 2], the severe form of which (α0-thalassemia) is characterized by the deletion of both pairs of linked α-globin genes, whereas a single α-gene deletion is present in individuals with α+-thalassemia
IC strip tests for α0-thalassemia (–Southeast Asian (SEA)) differential diagnosis
The anti-z-globin chain monoclonal antibody (mAb) PL2 and PL3 or the isotype-matched control mAb were first biotinylated using EZ-LinkTM Sulfo-NHS-LC-Biotin according to IC strip tests for α0-thalassemia (–SEA) differential diagnosis manufacturer instructions
Summary
Introduction αThalassemia is a genetic disorder caused by a defect in the α-globin gene [1, 2], the severe form of which (α0-thalassemia) is characterized by the deletion of both pairs of linked α-globin genes, whereas a single α-gene deletion is present in individuals with α+-thalassemia. Hb Bart’s hydrops fetalis is the most severe type of thalassemia and causes fetuses die in utero. Their mothers often suffer from several obstetric complications and must cope with the psychological burden of carrying a nonviable fetus to term [6, 7]. Polymerase chain reaction (PCR) is currently the most commonly used technique to diagnose α0-thalassemia [13,14,15,16]. This technique is not widely employed in routine laboratories of rural hospitals in resource-limited countries. The development of more cost effective and simplified techniques for identifying α0-thalassemia carriers are greatly needed for incorporation into the routine thalassemia screening programs of health promotion policies
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
