Development of lens opacities with peculiar characteristics in patients affected by thalassemia major on chelating treatment with deferasirox (ICL670) at the Pediatric Clinic in Monza, Italy

Masera, Nicoletta; Rescaldani, C.; Azzolini, Michele; Vimercati, Chiara; Tavecchia, L.; Masera, Giuseppe; Molfetta, V. De; Arpa, P.

doi:10.3324/haematol.11782

Cited by 12 publications

(10 citation statements)

References 6 publications

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“…Based on our results, no significant relationship was detected between retinal abnormalities and type of ICT. There are some disputes in the literature regarding the relationship between ICT and the occurrence of ocular manifestations [ 4 , 29 – 33 ]. Although ocular toxicity might be associated with administration of high-dose deferoxamine [ 34 ], it is usually reversible after drug interruption [ 35 , 36 ].…”

Section: Discussionmentioning

confidence: 99%

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran

et al. 2020

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Background Ocular involvement may occur via several mechanisms in patients with transfusion-dependent β-thalassemia (TDT) mainly chronic anemia, iron overload and iron chelator toxicity. We aimed to evaluate the frequency of abnormal ocular findings and their relationship with hematologic parameters in TDT patients. Methods In this cross-sectional study from January 2018 to January 2019, a total of 79 patients with TDT over the age of 18 who were on iron-chelation therapy (ICT) were consecutively investigated. All patients were registered at the Thalassemia Comprehensive Center affiliated with Shiraz University of Medical Sciences, Shiraz, Southern Iran. Complete ophthalmic examination was performed by an expert ophthalmologist. Clinical and hematologic parameters were collected from the patients´ medical records. Results The mean age ± standard deviation (SD) of the patients was 28.4 ± 5.6 years (range: 18–43). Twenty-four patients (30.4%) were male and 29 (36.7%) were splenectomized. The mean ± SD of the best-corrected visual acuity (VA) was 0.960 ± 0.086 decimal, (range: 0.6–1), 0.016 ± 0.046 logMar, (range: 0–0.2). The frequency of patients with VA > 0.1 logMar was 3 (3.8%). The mean intraocular pressure (IOP) was 14.88 ± 3.34 (6–25) mmHg. Fundus abnormalities were observed in 8 patients (10.1%), consisting of increased cup-disk ratio (3.8%), vessel tortuosity (2.5%), retinal pigment epithelium degeneration (2.5%), myelinated nerve fiber layer (1.3%), and internal limiting membrane wrinkling (1.3%). No significant association was observed between fundus abnormalities, VA, or IOP with hematologic parameters (P > 0.05). TDT patients with diabetes mellitus had significantly higher IOP (P = 0.010) but similar frequency of fundus abnormalities with non-diabetic patients (P > 0.05). Conclusions The frequency of ocular abnormalities in our patients was lower than the previous reports. The frequency of fundus abnormalities were similar in diabetic and non-diabetic thalassemia patients indicating close monitoring and proper management of the disease and comorbidities in these patients.

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Section: Discussionmentioning

confidence: 99%

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran

et al. 2020

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“…In our case, hyperferritinemia due to upregulated FPN function and additional factors inducing aberrant ferritin subunit synthesis, such as predominant FTL or imbalance of FTL/FTH, may promote the accumulation of ferritin in the lens and lead to early cataract formation. In addition, although our patient was followed by only phlebotomy, iron-chelating therapy with deferoxamine and deferasirox is considered to be a risk factor for cataracts (15,16), although the mechanism of pathogenesis has not been clarified.…”

Section: Discussionmentioning

confidence: 94%

A Novel Phenotype of a Hereditary Hemochromatosis Type 4 with Ferroportin-1 Mutation, Presenting with Juvenile Cataracts

Yamakawa

Yukawa

et al. 2016

Intern. Med.

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Hereditary hemochromatosis (HH) is an inherited disorder usually seen in Northern Europeans, which results in iron overload syndrome. A few cases have also been reported in Japan. We herein report a Japanese man presenting with fever, arthritis, liver dysfunction, and hyperferritinemia who was diagnosed with type 4 HH. He was heterozygous for the 1520A>G (His507Arg) mutation in the ferroportin-1 gene (SLC40A1). He had a familial cataract as an infant, but hereditary hyperferritinemia cataract syndrome was excluded. This is the first report of type 4 HH with juvenile cataracts and suggests that there is an association between hyperferritinemia and early cataract formation.

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“…However, five studies found no correlation between treatment and lens alterations 41 , 45 or, the observed prevalence was not different from the one reported in the general population. 30 , 31 , 42 , 43 Three case reports described four cases of lens opacities, three of which associated with deferiprone intake 18 , 19 and one with high intravenous doses of deferoxamine. 20 …”

Section: Resultsmentioning

confidence: 99%

“…Furthermore, it has not yet been established whether ophthalmological complications may be related to the dosage of chelating agents or their intake time. The majority of articles refer to deferoxamine, 16 , 20–41 while data about deferiprone 18 , 19 , 32 and deferasirox 42 , 43 are poor and do not allow to draw any conclusions.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Effects of Iron Chelating Agents on Ocular Function in Patients with Thalassemia Major

Nuzzi¹,

Geronazzo²,

Tridico³

et al. 2021

OPTH

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Background:The aim of this study is to evaluate eye structures and function in patients receiving iron chelating therapy and to assess whether a correlation exists between the onset of ocular alterations and the intake of iron chelating drugs. Methods: A prospective cohort study was performed. Eighty-eight patients, composed of children and adults with thalassemia major (TM) who are taking or had taken iron chelating drugs (deferoxamine, deferiprone or deferasirox), have been initially enrolled in the study. The final sample featured 80 patients, including 18 children and 62 adults. These subjects received an eye examination to evaluate intraocular pressure (IOP), best corrected visual acuity (BCVA), the presence of refractive defects, cornea, anterior chamber, lens, fundus oculi, visual field and mean retinal nerve fiber layer (RNFL) thickness. Logistic regression model analysis was performed in order to assess any correlation. In addition, a literature search regarding the relation between iron chelating drugs and ocular adverse events was carried out to compare the results obtained with the evidence in the literature. Results: Logistic regression did not report a significant correlation between the intake of iron chelating drugs and the onset of anterior ocular segment alterations, lens opacities, retinal diseases, optical neuropathies, astigmatism, visual field and RNFL thickness defects. Logistic regression returned a statistically significant correlation between myopia and iron chelation therapy (p-value 0.04; OR 1.05) and also between presbyopia and total duration of therapy with deferoxamine (p-value 0.03; OR 1.21). Although intraocular pressure levels remained within the normal range, a significant correlation with the length of deferoxamine therapy has been found (p-value 0.002; association coefficient −0.12). A negative correlation between deferiprone and presbyopia has also been observed. Conclusion: Iron chelation therapy is not associated with severe visual function alterations. Limitation of deferoxamine treatment can help prevent ocular complications. Deferiprone and/or deferasirox may be preferable, especially in patients over age 40 years.

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Development of lens opacities with peculiar characteristics in patients affected by thalassemia major on chelating treatment with deferasirox (ICL670) at the Pediatric Clinic in Monza, Italy

Cited by 12 publications

References 6 publications

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran

A Novel Phenotype of a Hereditary Hemochromatosis Type 4 with Ferroportin-1 Mutation, Presenting with Juvenile Cataracts

Long-Term Effects of Iron Chelating Agents on Ocular Function in Patients with Thalassemia Major

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