噬血细胞性淋巴组织细胞增多症患者眼部受累特征分析

The clinical characteristic of ocular findings secondary to hemophagocytic lymphohistiocytosis

目的观察噬血细胞性淋巴组织细胞增多症(HLH)患者眼部受累的特征性表现。方法回顾性分析2020年9月至2021年5月首都医科大学附属北京友谊医院收治的5例HLH合并眼部受累患者的临床资料,整理患者全身检查和治疗情况,分析眼部检查结果。在中国知网、Pubmed、WOS、Embase和Medicine数据库中检索有关HLH患者眼部受累的相关文献,总结HLH眼部受累的特征性表现。结果5例HLH患者中1例为原发性HLH患者,3例为EB病毒感染继发性HLH,1例为弥漫大B细胞淋巴瘤继发HLH。4例患者表现为单眼或双眼的眼底局限性出血和渗出,1例男性患者表现为双眼浆液性视网膜神经上皮脱离。文献检索结果显示,中文数据库共检索到1篇有关HLH患者眼部受累的论文,外文数据库共检索到11篇论文,HLH主要累及患者视神经、视网膜和脉络膜。结论HLH眼部受累因起病隐匿,临床表现复杂多样,容易被漏诊或误诊,早期检查和有效的治疗可能会挽救视力,避免错过全身治疗时机。

Objective To describe the characterization of ocular changes secondary to hemophagocytic lymphohistiocytosis(HLH).Methods The clinical data of 5 patients with HLH complicated with ocular involvement treated in Beijing Friendship Hospital Affiliated to Capital Medical University from September 2020 to May 2021 were retrospectively analyzed,the patients′systemic examination and treatment were sorted out,and the ocular examination results were analyzed.The relevant literatures on ocular involvement of HLH patients were searched in China National Knowledge Infrastructure(CNKI),Pubmed,WOS,Embase and Medicine databases to summarize the characteristic manifestations of ocular involvement of HLH.Results Of the 5 patients,1 had primary HLH,3 were Epstein-Barr virus-associated HLH(EBV-HLH),and 1 had diffuse large B-cell lymphoma-associated HLH.4 patients showed unilateral or bilateral focal retinal hemorrhage and exudates.1 male patient performed bilateral retinal pigment epithelial detachment.The literature search results showed that a total of 1 paper on ocular involvement in HLH patients was retrieved from the Chinese database and 11 papers were retrieved from the foreign database.HLH mainly involved the optic nerve,retina and choroid.Conclusions The ocular involvement of HLH is easy to be missed or misdiagnosed because of its occult onset and complex and diverse clinical manifestations.Early examination and effective treatment may save vision and avoid missing the opportunity of systemic treatment.