Abstract
Purpose
It was aimed to evaluate the retinochoroidal microvascular alterations of pediatric beta-thalassemia patients and investigate the effect of blood transfusion on perfusion among transfusion-dependent thalassemia (TDT), by means of optical coherence tomography angiography (OCTA).
Methods
In this multicentered, prospective, cross-sectional study, 56 TDT, 14 non-TDT (NTDT), and 63 healthy children were evaluated. The vessel density (VD) in superficial capillary plexus (SCP), deep capillary plexus, radial peripapillary capillary network, choriocapillaris, and the foveal avascular zone area (FAZ) were evaluated by OCTA. Before and after transfusion values of the TDT group were compared, and correlations were made with blood values and iron accumulation.
Results
Foveal and parafoveal zones were significantly thinner among TDT patients, with larger FAZ area. Macula VD of SCP and ppVD was lowest in NTDT group. In the TDT group, a decrease in retinal nerve fiber thickness and ppVD values was detected after transfusion. A negative significant relationship was found between both hemoglobin (Hb), hematocrit (Htc), and ppVD.
Conclusions
OCTA provides a better insight into retinal and choriocapillaris vascular impairment influenced by tissue hypoxia and oxidative stress in different clinical phenotypes of beta-thalassemia.
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References
Liaska A, Petrou P, Georgakopoulos CD et al (2016) β-Thalassemia and ocular implications: a systematic review. BMC Ophthalmol 16(1):1–13
Viprakasit V, Ekwattanakit S (2018) Clinical classification, screening and diagnosis for thalassemia. Hematol Oncol Clin N Am 32(2):193–211
Modell B, Darlison M (2008) Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 86(6):480–487
Vichinsky EP (2005) Changing patterns of thalassemia worldwide. Ann N Y Acad Sci 1054:18–24
Rathod DA, Kaur A, Patel V et al (2004) Usefulness of cell counter-based parameters and formulas in detection of β-thalassemia trait in areas of high prevalence. Am J Clin Pathol 128(4):585–589
Ghone RA, Kumbar KM, Suryakar AN, Katkam RV, Joshi NG (2008) Oxidative stress and disturbance in antioxidant balance in beta thalassemia major. Indian J Clin Biochem 23(4):337–340
Taneja R, Malik P, Sharma M, Agarwal MC (2010) Multiple transfused thalassemia major: ocular manifestations in a hospital-based population. Indian J Ophthalmol 58(2):125–130
Arden GB, Wonke B, Kennedy C, Huehns ER (1984) Ocular changes in patients undergoing long-term desferoxamine treatment. Br J Ophthalmol 68(12):873–877
Arifoglu HB, Kucuk B, Duru N et al (2018) Assessing posterior ocular structures in β-thalassemia minor. Int Ophthalmol 38(1):119–125
Ikram MK, Ong YT, Cheung CY, Wong TY (2013) Retinal vascular caliber measurements: clinical significance, current knowledge and future perspectives. Ophthalmologica 229(3):125–136
Wong TY (2004) Is retinal photography useful in the measurement of stroke risk? Lancet Neurol 3(3):179–183
Wylȩgała A, Teper S, Dobrowolski D, Wylȩgała E (2016) Optical coherence angiography: a review. Medicine (United States) 95(41):e4907
de Carlo TE, Romano A, Waheed NK, Duker JS (2015) A review of optical coherence tomography angiography (OCTA). Int J Retin Vitr 1(1):1–15
Madill SA (2021) Transient visual loss in young females with crowded optic discs: a proposed aetiology. Neuroophthalmology 45(6):372–379
Neyzi O, Furman A, Bundak R, Gunoz H, Darendeliler F, Bas F (2006) Growth references for Turkish children aged 6 to 18 years. Acta Paediatr Int J Paediatr 95(12):1635–1641
Taher A, Bashshur Z, Shamseddeen WA et al (2006) Ocular findings among thalassemia patients. Am J Ophthalmol 142(4):704–705
Uzun F, Karaca EE, Yıldız Yerlikaya G, Fındık H, Akın M (2019) Retinal nerve fiber layer thickness in children with β-thalassemia major. Saudi J Ophthalmol 31(4):224–228
Ulusoy MO, Türk H, Kıvanç SA (2019) Spectral domain optical coherence tomography findings in Turkish sickle-cell disease and beta thalassemia major patients. J Curr Ophthalmol 31(3):275–280
Qi X, Lewin AS, Sun L, Hauswirth WW, Guy J (2007) Suppression of mitochondrial oxidative stress provides long-term neuroprotection in experimental optic neuritis. Investig Ophthalmol Vis Sci 48(2):681–691
Moncef B, Hafedh J (2008) Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia. Intern Med 47(12):1125–1128
Sorsdahl OS, Taylor PE, Noyes WD (1964) Extramedullary hematopoiesis, mediastinal masses, and spinal cord compression. JAMA J Am Med Assoc 189(5):343–347
Shiba T, Sato Y, Takahashi M (2007) Relationship between proliferative diabetic retinopathy and sleep-disordered breathing. Nihon Ganka Gakkai Zasshi 111(11):899–904
Margolis R, Spaide RF (2009) A pilot study of enhanced depth imaging optical coherence tomography of the choroid in normal eyes. Am J Ophthalmol 147(5):811–815
AttaAllah HR, Mousa SO, Omar IAN (2021) Macular microvascular changes in children with transfusion-dependent beta-thalassemia. Graefes Arch Clin Exp Ophthalmol 259(11):3283–3293
Mathew R, Bafiq R, Ramu J et al (2015) Spectral domain optical coherence tomography in patients with sickle cell disease. Br J Ophthalmol 99(7):967–972
Cennamo G et al (2021) Retinal and choriocapillaris vascular changes in patients affected by different clinical phenotypes of beta-thalassemia: an optical coherence tomography angiography study. Biology (Basel) 10(4):276
Georgalas I, Makris G, Papaconstantinou D et al (2019) A pilot optical coherence tomography angiography study on superficial and deep capillary plexus foveal avascular zone in patients with beta-thalassemia major. Investig Ophthalmol Vis Sci 60(12):3887–3896
Song D, Dunaief JL (2013) Retinal iron homeostasis in health and disease. Front Aging Neurosci 5:1–13
Korkmaz MF, Can ME, Kazancı EG (2020) Effects of iron deficiency anemia on peripapillary and macular vessel density determined using optical coherence tomography angiography on children. Graefe’s Arch Clin Exp Ophthalmol 258(9):2059–2068
Barben M, Bordonhos A, Samardzija M, Grimm C (2019) Hypoxia-regulated MicroRNAs in the retina. Adv Exp Med Biol 1185:413–417
Acer S, Balcı YI, Pekel G et al (2016) Retinal nerve fiber layer thickness and retinal vessel calibers in children with thalassemia minor. SAGE Open Med 4:205031211666168
Ikeda Y, Tajima S, Yoshida S et al (2011) Deferoxamine promotes angiogenesis via the activation of vascular endothelial cell function. Atherosclerosis 215(2):339–347
Galanello R, Origa R (2010) Beta-thalassemia. Orphanet J Rare Dis 5(1):1–15
Tang FY, Chan EO, Sun Z et al (2020) Clinically relevant factors associated with quantitative optical coherence tomography angiography metrics in deep capillary plexus in patients with diabetes. Eye Vis 7(1):1–11
Güler Kazancı E, Korkmaz MF, Can ME (2020) Optical coherence tomography angiography findings in young β-thalassemia patients. Eur J Ophthalmol 30(3):600–607
Telfer PT, Prestcott E, Holden S, Walker M, Hoffbrand AV, Wonke B (2000) Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major. Br J Haematol 110(4):971–977
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All authors contributed to the study conception and design. The study was designed by UG and NÜ. Data were provided by HNY, NYÖ, and UG. Data collection was performed by UG. The first draft of the manuscript was written by UG and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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Güvenç, U., Ünlü, N., Yaralı, H.N. et al. Does thalassemia truly cause microvascular changes without us noticing? An optical coherence tomography angiography study of the children with beta-thalassemia. Int Ophthalmol 43, 3755–3765 (2023). https://doi.org/10.1007/s10792-023-02786-z
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DOI: https://doi.org/10.1007/s10792-023-02786-z