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伴有出血性葡萄膜炎和渗出性视网膜脱离的朗格汉斯细胞组织细胞增多症

Langerhans cell histiocytosis with hemorrhagic uveitis and exudative retinal detachment.

作者信息

Sitaula Ranju Kharel, Khatri Anadi

机构信息

Uveitis Department, B.P. Koirala Lions Center for Ophthalmic Studies, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal.

Fellow of Vitreo-Retina Surgery, Lumbini Eye Institute, Siddharthanagar, Nepal.

出版信息

Int Med Case Rep J. 2018 Mar 26;11:65-68. doi: 10.2147/IMCRJ.S162038. eCollection 2018.

DOI:10.2147/IMCRJ.S162038
PMID:29618940
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5875406/
Abstract

Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. A fixed dilated pupil with exudative retinal detachment was present in the right eye and hemorrhagic iris nodules with hyphema and hypopyon were seen in the left eye. Intraocular pressure was 12 and 31 mmHg, respectively, in each eye. Ocular symptoms were preceded by fever with multiple skin rashes, subcutaneous nodules, and lymph node enlargement. The histopathological examination of skin and lymph node showed histiocytes positive for histiocytic S 100. He was treated with topical steroids and antiglaucoma eye drops along with intravenous vinblastine 6 mg and oral prednisolone (1 mg/kg). Hyphema and hypopyon were resolved, vision improved to 20/320 and 20/80, and intraocular pressure was under control. However, as multisystemic LCH has a poor prognosis, we were unable to save him. Hence, fatal conditions like LCH should also be considered in the differential diagnosis of any hemorrhagic uveitis.

摘要

朗格汉斯细胞组织细胞增多症(LCH)累及双眼的情况并不常见。一名15岁男孩出现右眼无痛性视力下降以及左眼疼痛性眼红,持续2周。视力分别为20/500和20/200。右眼存在固定散大瞳孔伴渗出性视网膜脱离,左眼可见出血性虹膜结节伴前房积血和前房积脓。双眼眼压分别为12 mmHg和31 mmHg。眼部症状出现之前有发热,伴有多处皮疹、皮下结节和淋巴结肿大。皮肤和淋巴结的组织病理学检查显示组织细胞的组织细胞S 100呈阳性。他接受了局部类固醇和抗青光眼眼药水治疗,同时静脉注射长春新碱6 mg和口服泼尼松龙(1 mg/kg)。前房积血和前房积脓消退,视力改善至20/320和20/80,眼压得到控制。然而,由于多系统LCH预后较差,我们未能挽救他的生命。因此,在任何出血性葡萄膜炎的鉴别诊断中也应考虑LCH等致命疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/78e8e1c153d4/imcrj-11-065Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/120a70bc0af0/imcrj-11-065Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/49065180f38b/imcrj-11-065Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/87e89f364bd8/imcrj-11-065Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/78e8e1c153d4/imcrj-11-065Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/120a70bc0af0/imcrj-11-065Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/49065180f38b/imcrj-11-065Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/87e89f364bd8/imcrj-11-065Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e9b6/5875406/78e8e1c153d4/imcrj-11-065Fig4.jpg

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