Original Article

Hematologic Parameters Cut-off Assessment of Adult Alpha-thalassemia Patients in Iran

Hematologic Cut-Off Assessment of Alpha-Thalassemia

Abstract

Background: Thalassemia is one of the most common blood disorders in Iran. Alpha-thalassemia is caused by the deletion of the alpha-globin gene. The frequency of deletions in the alpha-globin gene is associated with microcytosis and hypochromia, making hematological parameters valuable predictive tools in the initial identification of alpha-thalassemia patients. This study aimed to compare hematologic parameters such as Mean Corpuscular Volume (MCV), Mean Corpuscular Hemoglobin (MCH), red blood cell (RBC) count, and hemoglobin (HGB) levels in silent and minor patients, whose genotypes were genetically characterized, with normal patients to establish cut-off points for these groups.

Materials and Methods: The study involved a total of 860 patients with alpha-thalassemia, including 267 cases of silent, 261 cases of minor, and 332 cases of normal alpha-thalassemia.

Results: Analysis of blood indices based on sex revealed that the male group had higher values than the female group. Assessment of alpha-thalassemia in minor patients showed that the Cis form (—/αα) had higher microcytosis than the Trans form (–α/–α) in this group. This difference was also observed between α-3.7α/ α-3.7α and (αα)-MED/αα as two different genetic forms in minor patients, with (αα)-MED/αα being in the Cis form. Data indicated that the cut-off value was insignificant in silent patients compared to the normal group. However, minor patients with MCH≤23.7 and MCV≤74.9 had an AUC greater than 0.9 (p-value< 0.01), distinguishing them from the normal group.

Conclusion: Comparing hematological parameters in these groups illustrated that MCV and MCH are the best predictor parameters for distinguishing between groups.

1. Dong BQ, Chen BY, Liang QY, et al. Study on the characteristics of major birth defects in 1.69 million cases of fetus in Guangxi Zhuang Autonomous Region. Zhonghua Liu Xing Bing Xue Za Zhi. 2019;40(12):1554-1559.
2. Muncie JR HL Campbell J. Alpha and beta thalassemia. Am Fam Physician. 2009;80(4):339-44.
3. Akhavan-Niaki H, Youssefi Kamangari R, Banihashemi A, et al. Hematologic features of alpha thalassemia carriers. Int J Mol Cell Med. 2012;1(3):162-7.
4. Nasiri A, Rahimi Z, Vaisi-Raygani A. Hemoglobinopathies in Iran: an updated review. Int J Hematol Oncol Stem Cell Res. 2020;14(2):140-150.
5. Karakaş Z, et al. Evaluation of alpha-thalassemia mutations in cases with hypochromic microcytic anemia: the Istanbul perspective. Turk J Hematol. 2015. 32(4):344-50.
6. Weatherall, D. and J. Clegg, The Thalassaemia Syndromes, Black well Scientific Publications. 1981, Oxford England.
7. Modell, B. and V. Berdoukas, The Clinical Approach to Thalassemia. London: Grune and Stratton. 1984, Harcourt Brace Jovanovich Inc.
8. Velasco-Rodríguez D, Blas C, Alonso-Domínguez JM, et al. Cut-off values of hematologic parameters to predict the number of alpha genes deleted in subjects with deletional alpha thalassemia. Int J Mol Sci. 2017; 18(12): 2707.
9. Anselmo FC, Ferreira NS, da Mota AJ, et al. Deletional alpha-thalassemia alleles in amazon blood donors. Adv Hematol. 2020; 2020: 4170259.
10. Motiani A, Zubair M, Sonagra AD. Laboratory Evaluation of Alpha Thalassemia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan. 2024 Feb 9.
11. Nunchai C, Sirichotiyakul S, Tongsong T. Optimal cutoff of mean corpuscular volume (MCV) for screening of alpha‐thalassemia 1 trait. J Obstet Gynaecol Res. 2020;46(5):774-778.
12. Idris F, Liew CY, Seman Z, et al. Optimal Mean Corpuscular Haemoglobin (MCH) Cut-Off Value for Differentiating Alpha Plus and Alpha Zero Thalassaemia in Thalassaemia Screening. Mal J Med Health Sci. 2020; 16(SUPP9): 69-74.
13. Doosti-Irani A, Cheraghi Z, Bitaraf S, et al. Prevalence of alpha and beta-thalassemia mutations among carriers of thalassemia in Shadegan city, southwest of Iran. Zahedan J Res Med Sci. 2015; 17(8):29-32.
14. Ahmad R, Saleem M, Aloysious NS, et al. Distribution of alpha thalassaemia gene variants in diverse ethnic populations in Malaysia: data from the Institute for Medical Research. Int J Mol Sci. 2013;14(9):18599-614.
15. Barrera‐Reyes, PK, Tejero ME. Genetic variation influencing hemoglobin levels and risk for anemia across populations. Ann N Y Acad Sci. 2019;1450(1):32-46.
Files
IssueVol 18 No 4 (2024) QRcode
SectionOriginal Article(s)
Keywords
Alpha thalassemia; Anemia; Mean corpuscular volume (MCV); Mean corpuscular hemoglobin (MCH)
Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Keikhaei B, Bitaraf S, Mafakher L, Galehdari H, Saki-Malehi A, Bahadoram M. Hematologic Parameters Cut-off Assessment of Adult Alpha-thalassemia Patients in Iran. Int J Hematol Oncol Stem Cell Res. 2024;18(4):324-330.