Service de Médecine Interne, Centre de Référence Maladies systémiques auto-immunes rares d'Ile de France, Hôpital Cochin, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Service d'ophtalmologie, Hôpital Cochin, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Surv Ophthalmol. 2021 Jan-Feb;66(1):124-131. doi: 10.1016/j.survophthal.2020.05.001. Epub 2020 May 11.
To identify clinical presentations, main causes, and prognosis of ophthalmic involvement in chronic lymphocytic leukemia (CLL), we performed a systematic review of articles describing CLL ophthalmic involvement in January 2019, using the PubMed database. We found 86 articles describing 123 cases of patients with ophthalmic involvement associated with CLL. Ophthalmic symptoms were CLL's first manifestation in 25.6% of patients and revealed Richter transformation in 11.0%. There were three main causes of ophthalmic features: CLL-infiltration (52.0%), lymphoma (26.0%), and infection (15.4%), with specific clinical and radiological characteristics. CLL-infiltration was mostly bilateral, whereas lymphoma was usually unilateral (P = 0.02). Optic neuropathy was always secondary to CLL-infiltration, and in those cases, cerebrospinal fluid immunophenotyping was a potential alternative to invasive biopsy as it confirmed the diagnosis in 4 patients (36.4%). On the contrary, lymphoma usually presented as adnexal involvement (P = 0.04), particularly as an orbital mass (P = 0.004). Infections concerned mostly patients previously treated for CLL (P < 0.0001), and main presentations included posterior uveitis (P = 0.0002) and retinal infiltrates (P < 0.0001). Overall, the prognosis was poor, as 29.3% of the patients died within 36 months of follow-up, and 26.1% had a partial or total visual loss. Eye infections were associated with the poorest prognosis as 47% of patients died, with a 6-month-median survival.
为了确定慢性淋巴细胞白血病（CLL）眼部受累的临床表现、主要原因和预后，我们于 2019 年 1 月在 PubMed 数据库中进行了系统回顾，以查找描述 CLL 眼部受累的文章。我们发现有 86 篇文章描述了 123 例 CLL 相关眼部受累患者。眼部症状是 CLL 患者的首发症状，占 25.6%，11.0%的患者出现 Richter 转化。眼部表现的主要原因有三种：CLL 浸润（52.0%）、淋巴瘤（26.0%）和感染（15.4%），具有特定的临床和影像学特征。CLL 浸润多为双侧，而淋巴瘤多为单侧（P = 0.02）。视神经病变总是继发于 CLL 浸润，在这些情况下，脑脊液免疫表型分析是一种替代有创性活检的潜在方法，因为它在 4 例患者中（36.4%）证实了诊断。相反，淋巴瘤通常表现为附属器受累（P = 0.04），尤其是眶内肿块（P = 0.004）。感染主要发生在之前接受过 CLL 治疗的患者中（P < 0.0001），主要表现为后葡萄膜炎（P = 0.0002）和视网膜浸润（P < 0.0001）。总体而言，预后较差，29.3%的患者在随访 36 个月内死亡，26.1%的患者有部分或完全视力丧失。眼部感染与最差的预后相关，47%的患者死亡，中位生存时间为 6 个月。
