An overview on thalassemia and challenges during COVID-19
Keywords:
Thalassemia, COVID-19, Beta thalassemia, Alpha thalassemiaAbstract
The thalassemia is a group of disorders in which the normal ratio of alpha globin to beta globin production is disrupted due to a disease-causing variant in one or more of the globin genes. This abnormal alpha- to beta-chain ratio causes the unpaired chains to precipitate and causes destruction of red blood cell precursors in the bone marrow (ineffective erythropoiesis) and circulation (hemolysis). Affected individuals with thalassemia have variable degrees of anemia and extramedullary hematopoiesis, which in turn can cause bone changes, impaired growth, and iron overload. The aim and objectives are to describe the basic genetic differences between alpha-thalassemia and beta-thalassemia. Describe the hematologic findings and pathophysiological changes that are associated with beta-thalassemia major. Summarize the etiology of thalassemias and describe the basic genetic differences between alpha-thalassemia and beta-thalassemia. Describe the genetic, hematologic, and clinical differences between alpha-thalassemia trait, hemoglobin H disease, and hydrops fetalis. outline the challenges of thalassemia during Covid 19 crisis. Thalassemia is the most common, inherited, single-gene disorder in the world. Treatment of the inherited blood disorder thalassemia depends upon the level of severity.
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Amani Abu-Shaheen, Humariya Heena, Abdullah Nofal, Doaa A. Abdelmoety, Abdulrahman Almatary, Mohammed Alsheef, Isamme AlFayyad, Epidemiology of Thalassemia in Gulf Cooperation Council Countries: A Systematic Review, BioMed Research International, vol. 2020, Article ID 1509501, https://doi.org/10.1155/2020/1509501. 2020; 15 pages.
Bajwa H, Basit H. Thalassemia. [Updated]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK545151/2021 Nov 5.
Apidechkul T, Yeemard F, Chomchoei C, Upala P, Tamornpark R Epidemiology of thalassemia among the hill tribe population in Thailand. PLoS ONE https://doi.org/10.1371/journal.pone.0246736. 2021; 16(2): e0246736.
Turgeon, Mary Louise. Clinical Hematology: Theory and Procedures. 5th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2012.
Al-Zwaini I. J. Introductory Chapter: Thalassemia - An Overview. In: AL-Zwaini, I. editor. Thalassemia and Other Hemolytic Anemias [Internet]. London: IntechOpen; 2018 [cited 2022 Mar 19]. Available from: https://www.intechopen.com/chapters/61536 doi: 10.5772/intechopen.77375.
Li CK. New trend in the epidemiology of thalassaemia. Best Pract Res Clin Obstet Gynaecol. doi: 10.1016/j.bpobgyn.2016.10.013. Epub 2016 Oct 26. PMID: 27847257. 2017 Feb; 39:16-26.
Sarah J. Kilpatrick and Sumire Kitahara. Anemia and Pregnancy. Creasy and Resnik's Maternal-Fetal Medicine: Principles and Practice, 55; 991-1006.e3.
Nienhuis, Arthur W, and David G Nathan. Pathophysiology and Clinical Manifestations of the β-Thalassemias. Cold Spring Harbor perspectives in medicine. doi:10.1101/cshperspect. a011726. 1 Dec. 2012; 2(12): a011726.
Weatherall DJ, Clegg JB. The thalassemia syndromes, 4th ed. Blackwell Science, Oxford 2001.
Thein SL Is it dominantly inherited β thalassemia or just a β-chain variant that is highly unstable? Br J Haematol 1999; 107: 12–21.
Galanello R, Cao A. Relationship between Genotype and Phenotype: Thalassemia Intermediaa. Annals of the New York Academy of Sciences. 1998; 850(1): 325-33.
Harewood J, Azevedo AM. Alpha Thalassemia. [Updated 2021 Sep 9]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; -. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441826. 2022 Jan.
Galanello R, Cao A. Gene test review. Alpha-thalassemia. Genet Med. doi: 10.1097/GIM.0b013e3181fcb468. PMID: 21381239. 2011 Feb;13(2):83-8.
Alpha Thalassemia Workup: Approach Considerations, Laboratory Studies, Hemoglobin Electrophoresis. emedicine.medscape.com. Retrieved 24 May 2016.
Tamary H, Dgany O. Alpha-Thalassemia. [updated 2020 Oct 1]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Mirzaa GM, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2022. PMID: 20301608. 2005 Nov 1.
Explore Thalassemias. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/thalassemia. July 2012.
Hassan M Yaish, MD. Pediatric Thalassemia. Medscape Reference. http://emedicine.medscape.com/article/958850-overview. April 2013.
Thalassemia. MedlinePlus. http://www.nlm.nih.gov/medlineplus/ency/article/000587.htm. February 2014.
Cianciulli P. Treatment of iron overload in thalassemia. Pediatr Endocrinol Rev. PMID: 19337180. 2008 Oct; 6 (1): 208-13.
Vogiatzi MG, Macklin EA, Fung EB, Cheung AM, Vichinsky E, Olivieri N, Kirby M, Kwiatkowski JL, Cunningham M, Holm IA, Lane J, Schneider R, Fleisher M, Grady RW, Peterson CC, Giardina PJ; Thalassemia Clinical Research Network. Bone disease in thalassemia: a frequent and still unresolved problem. J Bone Miner Res. doi: 10.1359/jbmr.080505. PMID: 18505376; PMCID: PMC3276604. 2009 Mar; 24(3): 543-57.
Symptoms and causes – Enlarged spleen (splenomegaly) – Mayo Clinic. www.mayoclinic.org. Archived from the original on 19 November 2016. Retrieved 2 February 2017.
Soliman AT, De Sanctis V, Kalra S. Anemia and growth. Indian J Endocrinol Metab.. doi: 10.4103/2230-8210.145038. PMID: 25538873; PMCID: PMC4266864. 2014 Nov;18(1): S1-5.
Thalassemia Complications. Thalassemia. Open Publishing. Archived from the original on 3 October 2011. Retrieved 27 September 2011.
Tari, K., Valizadeh Ardalan, P., Abbaszadehdibavar, M., Atashi, A., Jalili, A., Gheidishahran, M. Thalassemia an update: molecular basis, clinical features and treatment. International Journal of Biomedicine and Public Health, doi: 10.22631/ijbmph.2018.56102. 2018; 1(1): 48-58.
Okada S, Taketa K, Ishikawa T, Koji T, Swe T, Win N, et al. High prevalence of hepatitis C in patients with thalassemia and patients with liver diseases in Myanmar (Burma). Acta Medica Okayama. 2000; 54(3): 137-8.
Munkongdee T, Chen P, Winichagoon P, Fucharoen S and Paiboonsukwong K. Update in Laboratory Diagnosis of Thalassemia. Front. Mol. Biosci. 7:74. doi: 10.3389/fmolb.2020.00074. 2020.
Mohamed, Alkhansa Osama, Khalda Mirghani Hamza, and A. M. Babker. Physiological changes in some hematological and coagulation profile among Sudanese healthy pregnant women. Int J Med Sci Public Health 5.3 (2016); 525-8.
Singha K, Taweenan W, Fucharoen G, Fucharoen S. Erythrocyte indices in a large cohort of β-thalassemia carrier: Implication for population screening in an area with high prevalence and heterogeneity of thalassemia. Int J Lab Hematol. 2019 Aug; 41(4): 513-518.
Lynch EC. Peripheral Blood Smear. In: Walker HK, Hall WD, Hurst JW, editors. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition. Boston: Butterworths; Chapter 155. Available from: https://www.ncbi.nlm.nih.gov/books/NBK263/1990.
Lyashenko V.V., Babker A.M. and Lyubchenko V.A., The study of blood smear as the analysis of images of various objects. Medicine, 2017; 6(1): 17-21.
Babker AM. The Role of Erythroferrone Hormone as Erythroid and Iron Regulator in Several Hematological Disorders. Highlights on Medicine and Medical Research https://doi.org/10.9734/bpi/hmmr/v3/2493E. 2021; 3: 156–161.
Tatu, T. Laboratory Diagnosis of β-Thalassemia and HbE. In: Zakaria, M. Hassan, T. editors. Beta Thalassemia [Internet]. London: IntechOpen; [cited 2022 Mar 21]. Available from: https://www.intechopen.com/chapters/70780 doi: 10.5772/intechopen.90317. 2020
Nigam N, Singh P K, Agrawal M, et al. (September 30, 2020) MTHFR C677T, Prothrombin G20210A, and Factor V Leiden (G1691A) Polymorphism and Beta-Thalassemia Risk: A Meta-Analysis. Cureus 12(9): e10743. doi:10.7759/cureus.10743
Kahn JE, Veyssier-Belot C, Renier JL, de Mazancourt P, Peltier JY, de Raucourt E: Recurrent thromboembolism in a patient with beta-thalassemia major associated with double heterozygosity for factor V R506Q and prothrombin G20210A mutations. Blood Coagul Fibrinolysis. 2002, 13:461-463. 10.1097/00001721-200207000-00012
Babker, A.M.A.A.A. and Gameel, F.E.M.H., 2015. Molecular Characterization of Prothrombin G20210A gene Mutations In pregnant Sudanese women with spontaneous recurrent abortions. Rawal Medical Journal, 40(2), pp.207-209.
Babker, A. M. A. A. A., & Gameel, F. E. M. H. (2016). Methylenetetrahydrofolate reductase c677t polymorphism in Sudanese women with recurrent spontaneous abortions. Kuwait Medical Journal, 48(2), 100-104
Babker, A. M. (2020). The role of Inherited Blood Coagulation Disorders in Recurrent Miscarriage Syndrome. Journal of Critical Reviews, 7(1), 16-20.
Dharmesh Chandra Sharma, Anita Arya, Purnima Kishor, Poonam Woike, Jyoti Bindal. OVERVIEW ON THALASSEMIAS: A REVIEW ARTICLE. Med. res. chronicles [Internet]. 2017Jun.30 [cited 2022Mar.21]; 4(03): 325-37.
Malaz Faroug Mohamed, Albara Ahmad, Osman Mohammed Elmahi, Asaad Ma Babker and Hisham Ali Waggiallah, 2021. Susceptibility of Blood Groups Infection with COVID-19 Disease Among Sudanese Patients Suffering from Different Chronic Diseases. Pakistan Journal of Biological Sciences, 24: 815-820.
Abdullah I. Aedh AI, Al Hajri AH, Alshahrani AS, Adam MA, Dahab AA, Babker AMA, Mohamed H. Major Inflammatory Markers and Their Significance in Predicting Severity of COVID-19 Disease Pattern. International Journal of Biomedicine. doi:10.21103/Article11(4) OA13. 2021; 11(4): 488-492.
Aedh I. A., et al. Evaluating of the Clinical Profile, Laboratory Parameters and Outcome of Covid-19 Patients in Najran City-Saudi Arabia: A Descriptive Analysis. Journal of Pharmaceutical Research International (2021): 53-60.
Vij R, Machado RF. Pulmonary complications of hemoglobinopathies. Chest. 138:973–83. doi: 10.1378/chest.10-0317. 2010.
Lee JX, Chieng WK, Lau SCD and Tan CE COVID-19 and Hemoglobinopathies: A Systematic Review of Clinical Presentations, Investigations, and Outcomes. Front. Med. 8:757510. doi: 10.3389/fmed.2021.757510. 2021.
De Sanctis V, Canatan D, Corrons JLV, et al. A comprehensive update of ICET-A Network on COVID-19 in thalassemias: what we know and where we stand. Acta Biomed. Published 2020 Sep 7. doi:10.23750/abm. v91i3.10063. 2020; 91(3): e2020026.
Okar L.; Ali, M.; Parengal J.; Yassin M.A. COVID-19 and thalassemia beta major in splenectomized patient: Clinical case progression and literature review. Clin. Case Rep. 2020: 8; 2918–2922.
Hossain M.S., Raheem E. & Siddiqee M.H. The forgotten people with thalassemia in the time of COVID-19: South Asian perspective. Orphanet J Rare Dis https://doi.org/10.1186/s13023-020-01543-0. 2020; 15: 265.
Oymak Y, Karapinar TH. COVID-19 Pandemic and Thalassemia Major Patients: Transfusion Practice and Treatment Assessment. J Pediatr Hematol Oncol. doi: 10.1097/MPH.0000000000002016. PMID: 33273415. 2021 Nov 1; 43(8): e1073-e1076.
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