Angioid Streaks in Homozygous β Thalassemia

Aessopos, Athanasios; Stamatelos, G.; Savvides, P.; Kavouklis, E.; Gabriel, L; Rombos, Ioannis; Karagiorga, Markisia; Kaklamanis, Phaedon

doi:10.1016/s0002-9394(14)73300-8

Cited by 32 publications

(14 citation statements)

References 11 publications

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“…16 In ␤ thalassemia, angioid streaks were initially described in isolated cases [18][19][20] and subsequently, an occurrence of 20% was reported in a series of 100 patients ( Figure 1). 21 Being manifested after the age of 20 years, the findings were positively correlated with age also in this group. 21 Furthermore, angioid streaks have been encountered in sickle thalassemia 22 and a frequency of 10% was reported in a group of 58 cases.…”

Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Sysupporting

confidence: 61%

“…21 Being manifested after the age of 20 years, the findings were positively correlated with age also in this group. 21 Furthermore, angioid streaks have been encountered in sickle thalassemia 22 and a frequency of 10% was reported in a group of 58 cases. 23 The etiology of angioid streaks in these hemoglobinopathies has not been clarified.…”

Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Sysupporting

confidence: 61%

“…Impaired visual acuity due to subretinal neovascularization and hemorrhage has been described in 2 patients with thalassemia major and another with thalassemia intermedia. 18,21,80 Regarding the vascular involvement, it is a question whether it has a contributing role in the development of leg ulcerations, tibial cramps, and easy bruising, features that are frequent in thalassemic and SCD patients. In ␤ thalassemia, fatal intracranial hemorrhages have been described in 2 patients with PXE lesions.…”

Section: Pxe-related Complicationsmentioning

confidence: 99%

See 2 more Smart Citations

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Aessopos

Farmakis

Loukopoulos

2002

Blood

139

124

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The development of clinical and histopathologic manifestations of a diffuse elastic tissue defect, resembling inherited pseudoxanthoma elasticum (PXE), has been encountered with a notable frequency in patients with ␤ thalassemia, sickle cell disease, and sickle thalassemia. The PXE-like clinical syndrome, consisting of skin, ocular, and vascular manifestations, has a variable severity in these hemoglobinopathies and it is age-dependent, with a generally late onset, after the second decade of life. The defect is believed to be acquired rather than inherited and related to the consequences of the primary disease. The high prevalence of the findings implicates the elastic tissue injury as one of the main comorbid abnormalities encountered in ␤ thalassemia and the sickling syndromes. In these patients a number of complications, sometimes serious, has been recognized to be related to ocular and vascular elastic tissue defects. Because several organ systems are involved, each medical specialty should be aware of the phenomenon. This coexistence, on the other hand, introduces a novel pathogenetic aspect of PXE and an important research challenge. (Blood. 2002;99:30-35)

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Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Sysupporting

confidence: 61%

Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Sysupporting

confidence: 61%

Section: Pxe-related Complicationsmentioning

confidence: 99%

See 1 more Smart Citation

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Aessopos

Farmakis

Loukopoulos

2002

Blood

139

124

View full text Add to dashboard Cite

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“…Angioid streaks are thought to be milder in hemoglobinopathies than in inherited PXE and visual acuity impairment is generally considered to be uncommon. However, visual loss due to AS has already been described in two patients with TM, one with TI and one with sickle thalassemia [10][11][12][13], while severe reduction of visual acuity due to AS was encountered in both TI patients of this study. Of note is the absence of choroidal neovascularization in the first patient, showing that extended AS lesions alone are sufficient to cause loss of vision due to RPE atrophy, without allowing any further therapeutic intervention.…”

Section: Discussionsupporting

confidence: 46%

Loss of vision associated with angioid streaks in β-thalassemia intermedia

et al. 2007

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An acquired diffuse elastic tissue defect that resembles inherited pseudoxanthoma elasticum (PXE) has been noticed with a significant age-related frequency in hemoglobin disorders, especially beta-thalassemia and has been held responsible for a number of complications observed in these cases, some of which are quite severe. We report here two patients with beta-thalassemia intermedia, who presented with severe visual acuity impairment associated with angioid streaks, the typical ocular manifestation of PXE.

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“…The disease can be isolated or associated with systemic disorders such as pseudoxanthoma elasticum [4,5] , pseudoxanthoma elasticum-like syndromes [6] , β-thalassemia [7] , sickle cell disease [8] , and Paget disease [9] . The occurrence of choroidal neovascularization (CNV) in the subretinal space complicates the disease and frequently results in legal blindness, especially in middleaged patients.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Follow-Up of Choroidal Neovascularization due to Angioid Streaks with pro re nata Intravitreal Anti-VEGF Treatment

et al. 2017

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Purpose: To evaluate the long-term outcomes of intravitreal anti-vascular endothelial growth factor (VEGF) drugs with a pro re nata (PRN) regimen for the treatment of choroidal neovascularization (CNV) secondary to angioid streaks (AS). Methods: This is a retrospective, multicenter, noncomparative case series of consecutive AS eyes affected by treatment-naïve CNV. A complete ophthalmologic examination was performed every 30-45 days after the loading phase, including fluorescein angiography and/or optical coherence tomography. Results: In all, 52 eyes of 39 patients were treated with intravitreal bevacizumab and/or ranibizumab and followed up for a mean of 33.8 months. The best corrected visual acuity at baseline was 20/40, and it deteriorated by an average of 6.8 ETDRS letters per year (p < 0.001). We performed an average of 5.1, 6.5, and 6.8 injections at the 1-, 2-, and 3-year follow-up, respectively. Conclusions: Intravitreal anti-VEGF drugs in a PRN regimen with close monitoring appear to slow the progression of CNV in AS, but they do not prevent the affected eyes from progressive visual loss.

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Angioid Streaks in Homozygous β Thalassemia

Cited by 32 publications

References 11 publications

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Loss of vision associated with angioid streaks in β-thalassemia intermedia

Long-Term Follow-Up of Choroidal Neovascularization due to Angioid Streaks with pro re nata Intravitreal Anti-VEGF Treatment

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