Interaction of hemoglobin Grey Lynn (Vientiane) with a non-deletional α(+)-thalassemia in an adult Thai proband.

Abstract

Hemoglobin (Hb) Grey Lynn is a Hb variant caused by a substitution of Phe for Leu at position 91 of α1-globin chain, originally described in individual of unknown ethnic background. This article addresses the interaction of Hb Grey Lynn with a non-deletional α(+)-thalassemia found in Thailand, a hitherto un-described condition. The proband was adult Thai woman referred for investigation of mild anemia with Hb 90 g/L. Hb analyses using low pressure liquid chromatography raised a suspicion of abnormal Hb presence, which was failed to demonstrate by cellulose acetate electrophoresis and capillary electrophoresis. DNA sequencing identified a CTT (Leu) to TTT (Phe) mutation at codon 91 corresponding to the Hb Grey Lynn (Vientiane) [α91(FG3)Leu>Phe (α1) on α1-globin gene and a C deletion between codons 36 and 37 on α2-globin gene causing α(+)-thalassemia. As compared to those observed in a compound heterozygote for Hb Grey Lynn / α(0)-thalassemia reported previously, higher MCV (81.7 fL) and MCH (26.3 pg) values with a lower level of Hb Grey Lynn (19.7%) were observed in the proband. The normochromic normocytic anemia observed could be due to the interaction of Hb Grey Lynn with α(+)-thalassemia. The two mutations could be identified using PCR-RFLP and allele-specific PCR assays developed.

• Authors: Kritsada Singha, Goonnapa Fucharoen, Supan Fucharoen
• Journal: Biochemia medica (Biochem Med (Zagreb)) Vol. 24 Issue 1 Pg. 167-74 ( 2014) ISSN: 1330-0962 [Print] Croatia
• PMID: 24627726 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hemoglobins, Abnormal
• hemoglobin Grey Lynn

Topics

• Adult
• Base Sequence
• Female
• Gene Deletion
• Hemoglobins, Abnormal (genetics, metabolism)
• Heterozygote
• Humans
• Male
• Middle Aged
• Molecular Sequence Data
• Point Mutation (genetics)
• Polymerase Chain Reaction
• Polymorphism, Restriction Fragment Length
• Sequence Homology, Nucleic Acid
• Thailand
• alpha-Thalassemia (genetics, metabolism)