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Chronic refractory uveitis in a patient with childhood-onset cyclic neutropenia.

Chen LL, Toyoguchi M, Shimakawa M, Hori S - Case Rep Ophthalmol (2011)

Bottom Line: Her eyes were treated with topical corticosteroids, retinal photocoagulation, and cataract surgery.Blood and bone marrow test results confirmed the diagnosis of CN.She also fulfilled the diagnostic criteria of Behçet's disease, though clinical features of her uveitis were dissimilar to those found in that disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan.

ABSTRACT
We report a rare case of chronic refractory uveitis in a patient with childhood-onset cyclic neutropenia (CN). A 19-year-old woman, who had a history of CN beginning at age 2, presented with bilateral chronic nongranulomatous uveitis, complicated cataract, retinal vasculitis, cystoids macular edema, and vitreous hemorrhage. She had recurrent episodes of oral ulcers, tonsillitis, genital ulcers, and folliculitis during neutropenic nadir. After the resumption of granulocyte colony-stimulating factor therapy for her CN, vitreous hemorrhage in both eyes followed. Her eyes were treated with topical corticosteroids, retinal photocoagulation, and cataract surgery. Blood and bone marrow test results confirmed the diagnosis of CN. She also fulfilled the diagnostic criteria of Behçet's disease, though clinical features of her uveitis were dissimilar to those found in that disease.

No MeSH data available.


Related in: MedlinePlus

Fundus photography and fluorescein angiogram on initial examination. a, b Fundus photographs were notable for neovascularization of arcade vessels surrounding the optic disc, proliferative membrane, and cystoid macular edemas in the right eye (a) and in the left eye (b). c, d Fluorescein angiogram of the right eye (c) and the left eye (d) confirmed neovascularization and cystoid macular edemas in the posterior poles. e Diffuse leakage in varying degrees from retinal vessels indicating vasculitis in mid-periphery of the right eye. f Similar findings are seen in the left eye.
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Figure 1: Fundus photography and fluorescein angiogram on initial examination. a, b Fundus photographs were notable for neovascularization of arcade vessels surrounding the optic disc, proliferative membrane, and cystoid macular edemas in the right eye (a) and in the left eye (b). c, d Fluorescein angiogram of the right eye (c) and the left eye (d) confirmed neovascularization and cystoid macular edemas in the posterior poles. e Diffuse leakage in varying degrees from retinal vessels indicating vasculitis in mid-periphery of the right eye. f Similar findings are seen in the left eye.

Mentions: A 19-year-old woman with a history of childhood-onset CN was referred to our clinic in 1999. She had had red eyes from age 6 and had been diagnosed with uveitis at age 9. From age 9 to 17 she had received granulocyte colony-stimulating factor (G-CSF) therapy for her CN. She had been under topical corticosteroid therapy for more than 10 years. On initial examination, her best-corrected visual acuity was 20/60 OD and 20/100 OS. Both eyes had moderate nongranulomatous uveitis associated with band keratopathy and complicated cataract. Neovascularization of optic disc and arcade vessels, proliferative membrane, and cystoid macular edema were also present (fig. 1, top). Vitreous hemorrhage was seen in the left eye. Fluorescein angiography (fig. 1, middle and bottom) revealed diffuse leakage in varying degrees from retinal vessels. Indocyanine green angiography in the mid-periphery demonstrated multiple faint hypo-fluorescent spots. Blood and bone marrow test results were compatible with a diagnosis of CN. Peripheral white blood cell counts ranged from 3,300/ml with 0.5% neutrophils to 6,850/ml with 60.3% neutrophils. Neutrophil function, the oxidative burst and phagocytosis ability, and serum tumor necrosis factor level were normal in both nadir and peak of the cycling. Mutation in exon 4 of the neutrophil ELA2 gene [1] was detected in DNA extracted from peripheral blood. HLA-B51 was positive and pathergy test was negative. Recurrent oral ulcers, tonsillitis, genital ulcers, and folliculitis were noted; thus, she fulfilled the ISGBD criteria. However, these symptoms diminished when she wore an isolator, gargled thoroughly with povidone-iodine and amphotericin B, and ate only pasteurized food while in the hospital. In February 2000, she resumed G-CSF therapy. Four months later she had vitreous hemorrhage in both eyes and consequently her visual acuity became impaired (fig. 2, top). Because of this event, the hematologist stopped G-CSF therapy and switched to conservative treatment. Because the patient's left eye had four instances of vitreous hemorrhage and color Doppler imaging showed strangulation of central retinal vein, we performed retinal photocoagulation. Subsequently, neovascularization of optic disc and arcade vessels subsided in both eyes despite continued chronic uveitis. In May 2005, complicated cataract had deteriorated the visual acuity to 20/200 in both eyes. Cataract extraction with posterior chamber lens implantation resulted in stable visual acuity of 20/60 OD and 20/40 OS.


Chronic refractory uveitis in a patient with childhood-onset cyclic neutropenia.

Chen LL, Toyoguchi M, Shimakawa M, Hori S - Case Rep Ophthalmol (2011)

Fundus photography and fluorescein angiogram on initial examination. a, b Fundus photographs were notable for neovascularization of arcade vessels surrounding the optic disc, proliferative membrane, and cystoid macular edemas in the right eye (a) and in the left eye (b). c, d Fluorescein angiogram of the right eye (c) and the left eye (d) confirmed neovascularization and cystoid macular edemas in the posterior poles. e Diffuse leakage in varying degrees from retinal vessels indicating vasculitis in mid-periphery of the right eye. f Similar findings are seen in the left eye.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
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getmorefigures.php?uid=PMC3104861&req=5

Figure 1: Fundus photography and fluorescein angiogram on initial examination. a, b Fundus photographs were notable for neovascularization of arcade vessels surrounding the optic disc, proliferative membrane, and cystoid macular edemas in the right eye (a) and in the left eye (b). c, d Fluorescein angiogram of the right eye (c) and the left eye (d) confirmed neovascularization and cystoid macular edemas in the posterior poles. e Diffuse leakage in varying degrees from retinal vessels indicating vasculitis in mid-periphery of the right eye. f Similar findings are seen in the left eye.
Mentions: A 19-year-old woman with a history of childhood-onset CN was referred to our clinic in 1999. She had had red eyes from age 6 and had been diagnosed with uveitis at age 9. From age 9 to 17 she had received granulocyte colony-stimulating factor (G-CSF) therapy for her CN. She had been under topical corticosteroid therapy for more than 10 years. On initial examination, her best-corrected visual acuity was 20/60 OD and 20/100 OS. Both eyes had moderate nongranulomatous uveitis associated with band keratopathy and complicated cataract. Neovascularization of optic disc and arcade vessels, proliferative membrane, and cystoid macular edema were also present (fig. 1, top). Vitreous hemorrhage was seen in the left eye. Fluorescein angiography (fig. 1, middle and bottom) revealed diffuse leakage in varying degrees from retinal vessels. Indocyanine green angiography in the mid-periphery demonstrated multiple faint hypo-fluorescent spots. Blood and bone marrow test results were compatible with a diagnosis of CN. Peripheral white blood cell counts ranged from 3,300/ml with 0.5% neutrophils to 6,850/ml with 60.3% neutrophils. Neutrophil function, the oxidative burst and phagocytosis ability, and serum tumor necrosis factor level were normal in both nadir and peak of the cycling. Mutation in exon 4 of the neutrophil ELA2 gene [1] was detected in DNA extracted from peripheral blood. HLA-B51 was positive and pathergy test was negative. Recurrent oral ulcers, tonsillitis, genital ulcers, and folliculitis were noted; thus, she fulfilled the ISGBD criteria. However, these symptoms diminished when she wore an isolator, gargled thoroughly with povidone-iodine and amphotericin B, and ate only pasteurized food while in the hospital. In February 2000, she resumed G-CSF therapy. Four months later she had vitreous hemorrhage in both eyes and consequently her visual acuity became impaired (fig. 2, top). Because of this event, the hematologist stopped G-CSF therapy and switched to conservative treatment. Because the patient's left eye had four instances of vitreous hemorrhage and color Doppler imaging showed strangulation of central retinal vein, we performed retinal photocoagulation. Subsequently, neovascularization of optic disc and arcade vessels subsided in both eyes despite continued chronic uveitis. In May 2005, complicated cataract had deteriorated the visual acuity to 20/200 in both eyes. Cataract extraction with posterior chamber lens implantation resulted in stable visual acuity of 20/60 OD and 20/40 OS.

Bottom Line: Her eyes were treated with topical corticosteroids, retinal photocoagulation, and cataract surgery.Blood and bone marrow test results confirmed the diagnosis of CN.She also fulfilled the diagnostic criteria of Behçet's disease, though clinical features of her uveitis were dissimilar to those found in that disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, School of Medicine, Tokyo Women's Medical University, Tokyo, Japan.

ABSTRACT
We report a rare case of chronic refractory uveitis in a patient with childhood-onset cyclic neutropenia (CN). A 19-year-old woman, who had a history of CN beginning at age 2, presented with bilateral chronic nongranulomatous uveitis, complicated cataract, retinal vasculitis, cystoids macular edema, and vitreous hemorrhage. She had recurrent episodes of oral ulcers, tonsillitis, genital ulcers, and folliculitis during neutropenic nadir. After the resumption of granulocyte colony-stimulating factor therapy for her CN, vitreous hemorrhage in both eyes followed. Her eyes were treated with topical corticosteroids, retinal photocoagulation, and cataract surgery. Blood and bone marrow test results confirmed the diagnosis of CN. She also fulfilled the diagnostic criteria of Behçet's disease, though clinical features of her uveitis were dissimilar to those found in that disease.

No MeSH data available.


Related in: MedlinePlus