TEMPI综合征的眼部受累情况。
Ocular involvement in TEMPI syndrome.
作者信息
Wu Jo-Hsuan, Viruni Narine, Chun Justin, Shanbhag Satish, Liu T Y Alvin
机构信息
Shiley Eye Institute and Viterbi Family Department of Ophthalmology, University of California, San Diego, CA, 92093, USA.
Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, 21287, USA.
出版信息
Am J Ophthalmol Case Rep. 2022 Apr 10;26:101534. doi: 10.1016/j.ajoc.2022.101534. eCollection 2022 Jun.
PURPOSE
We report the first case of ocular involvement in TEMPI syndrome, a rare disease characterized by telangiectasias, elevated erythropoietin with erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intra-pulmonary shunting.
OBSERVATIONS
A 64-year-old Caucasian man with history of TEMPI syndrome presented with subacute bilateral painless vision loss. Ocular examination showed chronic retinal ischemia with microvascular damage, which was likely associated with the chronic systemic hypoxemia, and spontaneous wax and wane of cystoid macular edema, presumedly related to the systemic bortezomib treatment.
CONCLUSIONS AND IMPORTANCE
Our case demonstrates that pathologic retinal vascular changes could be seen in association with TEMPI syndrome and suggests that a comprehensive ophthalmological examination may be beneficial for these patients.
目的
我们报告了首例眼部受累的TEMPI综合征病例，这是一种罕见疾病，其特征为毛细血管扩张、促红细胞生成素升高伴红细胞增多、单克隆丙种球蛋白病、肾周积液和肺内分流。
观察结果
一名有TEMPI综合征病史的64岁白种男性出现亚急性双侧无痛性视力丧失。眼部检查显示慢性视网膜缺血伴微血管损伤，这可能与慢性全身性低氧血症有关，以及黄斑囊样水肿的自发消长，推测与全身性硼替佐米治疗有关。
结论与意义
我们的病例表明，与TEMPI综合征相关的病理性视网膜血管变化可见，提示全面的眼科检查可能对这些患者有益。
Zotero 插件