Literature DB >> 35496953

Erythrocyte Indices and Hemoglobin Analysis for α-Thalassemia Screening in an Area with High Carrying Rate.

Lin Zheng1, Hailong Huang1, Xiaoqing Wu1, Qingmei Shen1, Meihuan Chen1, Meiying Wang1, Linjuan Su1, Liangpu Xu1.   

Abstract

Carriers of α-thalassemia exhibit hypochromic microcytosis with mean corpuscular volume (MCV) < 80 fL, mean corpuscular hemoglobin (MCH) < 27 pg, and reduced hemoglobin A2 (HbA2). We studied the distribution and diagnostic efficiencies of these indicators and their combinations in patients with and without alpha-thalassemia. Based on genetic diagnosis, 10,883 participants were divided into alpha-thalassemia group (n = 1655) and negative-for-alpha-thalassemia group (n = 9228). Erythrocyte parameters and hemoglobin analysis of the groups were analyzed. Moreover, we compared the four screening schemes (MCV/MCH, MCV/MCH/HbA2, MCV + MCH, MCV + MCH + HbA2) to find the best for α-thalassemia screening. The genotypes of --SEA/αα, and -α3.7/αα are the most prevalent with 54.9% and 27.6% in Fujian Province, China. There were significant differences in the distribution of MCV, MCH, and HbA2 in the two groups. Among the three, MCH exhibited the highest sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy. Although the four screening schemes have their advantages, there are significant differences in their sensitivity and specificity. MCV + MCH had the best diagnostic performance (72.6% sensitivity, 89.0% specificity) as well as the highest Youden index (61.59%). Our results showed that MCH could be used to screen α-thalassemia instead of MCV and HbA2. However, it is recommended that MCV/MCH/HbA2 screening be used in areas with high α-thalassemia incidence to increased sensitivity. © Indian Society of Hematology and Blood Transfusion 2021.

Entities:  

Keywords:  Hemoglobin A2; Mean corpuscular hemoglobin; Mean corpuscular volume; α-thalassemia

Year:  2021        PMID: 35496953      PMCID: PMC9001776          DOI: 10.1007/s12288-021-01449-2

Source DB:  PubMed          Journal:  Indian J Hematol Blood Transfus        ISSN: 0971-4502            Impact factor:   0.900


  38 in total

1.  The effect of iron deficiency anaemia on the levels of haemoglobin subtypes: possible consequences for clinical diagnosis.

Authors:  I El-Agouza; A Abu Shahla; M Sirdah
Journal:  Clin Lab Haematol       Date:  2002-10

2.  Unusual rearrangement of the alpha-globin gene cluster containing both the -alpha3.7 and alphaalphaalphaanti-4.2 crossover junctions: clinical diagnostic implications and possible mechanisms.

Authors:  Wen Wang; Amy Y Y Chan; Li-Chong Chan; Edmond S K Ma; Samuel S Chong
Journal:  Clin Chem       Date:  2005-11       Impact factor: 8.327

Review 3.  Cure for thalassemia major - from allogeneic hematopoietic stem cell transplantation to gene therapy.

Authors:  Alok Srivastava; Ramachandran V Shaji
Journal:  Haematologica       Date:  2016-12-01       Impact factor: 9.941

Review 4.  New trend in the epidemiology of thalassaemia.

Authors:  Chi-Kong Li
Journal:  Best Pract Res Clin Obstet Gynaecol       Date:  2016-10-26       Impact factor: 5.237

5.  The spectrum of α- and β-thalassemia mutations of the Li people in Hainan Province of China.

Authors:  Hongxia Yao; Xinping Chen; Lie Lin; Congming Wu; Xiangjun Fu; Hua Wang; Zhiming Yao; Wenting Chen; Li Huang; Ruimei Tang; Ruo Rao; Suwen Wang; Yipeng Ding
Journal:  Blood Cells Mol Dis       Date:  2014-02-16       Impact factor: 3.039

Review 6.  Alpha-thalassaemia.

Authors:  Cornelis L Harteveld; Douglas R Higgs
Journal:  Orphanet J Rare Dis       Date:  2010-05-28       Impact factor: 4.123

Review 7.  Gene Therapy Approaches to Hemoglobinopathies.

Authors:  Giuliana Ferrari; Marina Cavazzana; Fulvio Mavilio
Journal:  Hematol Oncol Clin North Am       Date:  2017-10       Impact factor: 3.722

Review 8.  Molecular Basis and Genetic Modifiers of Thalassemia.

Authors:  Sachith Mettananda; Douglas R Higgs
Journal:  Hematol Oncol Clin North Am       Date:  2018-04       Impact factor: 3.722

9.  Molecular characterization of α- and β-thalassemia in the Yulin region of Southern China.

Authors:  Sheng He; Jihui Li; Dong Ming Li; Shang Yi; Xiongcai Lu; Yudi Luo; Yi Liang; Chunfeng Feng; Biyan Chen; Chenguang Zheng; Xiaoxia Qiu
Journal:  Gene       Date:  2018-02-23       Impact factor: 3.688

10.  The prevalence of thalassemia in mainland China: evidence from epidemiological surveys.

Authors:  Ketong Lai; Guifeng Huang; Li Su; Yunyan He
Journal:  Sci Rep       Date:  2017-04-19       Impact factor: 4.379

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