Lisch Walter, Vossmerbaeumer Urs
Department of Ophthalmology, University Medical Center of the Johannes Gutenberg- University Mainz, Mainz, Germany.
Am J Ophthalmol Case Rep. 2020 Sep 18;20:100926. doi: 10.1016/j.ajoc.2020.100926. eCollection 2020 Dec.
To report a female patient of biclonal Lewis syndrome which consists of a trias: biclonal gammopathy of undetermined significance, paraproteinemic keratopathy in form of a brownish discoid opacification at the level of Descemet's membrane and hypercupremia. After several years there was a conversion to multiple myeloma. Systemic chemotherapy led to a complete remission of multiple myeloma and to a normalization of the copper level in the blood that lasted five years. The corneal opacification remained unchanged.
A currently 66-year-old woman suffered from biclonal Lewis syndrome. On both eyes there is a central discoid yellow-brownish discoloration in the Pre-Descemet's layer. The corneal findings were unchanged after a follow up of eight years. However, there was a conversion to multiple myeloma (MM) type IgG with progressive anemia and suspicious bone lesions. A multiple systemic myeloma-therapy was indicated. Chemotherapy with subsequent tandem autologous-stem cell therapy (auto-SCT) was performed. The blood examination after this therapy showed a complete remission of multiple myeloma, and it was also very surprising that the serum copper level was within normal range. This finding remained unchanged over a period of five years. The bilateral corneal opacification was identical to that before chemotherapy. To the best of our knowledge, this represents the first observation of a normalization of copper levels in Lewis syndrome after chemotherapy.
The Lewis syndrome represents a very rare disorder. The first case reported in Europe (Lisch et al., 2016) showed a conversion from biclonal gammopathy of undetermined significance to MM after a follow-up of 17 years. Subjectively, the patient was in excellent health. The typical corneal, discoid brownish opacification at the level of Descemet's membrane is a suspicious hint of a copper disturbance for the ophthalmologist. The copper level of our patient was extremely elevated. The corneal opacification however, remained unchanged throughout the repeated ophthalmological examinations. In 2015, the conversion of our case with Lewis syndrome to MM performing chemotherapy in different steps and a twice auto-SCT resulted in a complete remission of MM and a normal range of the serum copper. The bilateral corneal opacification remained unchanged during an observation period of five years after chemotherapy.
报告一例双克隆刘易斯综合征女性患者，该综合征包括三联征：意义未明的双克隆丙种球蛋白病、以Descemet膜水平的褐色盘状混浊形式存在的副蛋白血症性角膜病变以及高铜血症。数年后转变为多发性骨髓瘤。全身化疗导致多发性骨髓瘤完全缓解，血液中铜水平恢复正常并持续了五年。角膜混浊保持不变。
一名现年66岁的女性患有双克隆刘易斯综合征。双眼在Descemet膜前层有中央盘状黄棕色变色。随访8年后角膜病变无变化。然而，转变为IgG型多发性骨髓瘤（MM），伴有进行性贫血和可疑骨病变。需要进行多次全身骨髓瘤治疗。进行了化疗及随后的串联自体干细胞治疗（auto-SCT）。该治疗后的血液检查显示多发性骨髓瘤完全缓解，同样令人惊讶的是血清铜水平在正常范围内。这一发现持续五年未变。双侧角膜混浊与化疗前相同。据我们所知，这是化疗后刘易斯综合征患者铜水平恢复正常的首例观察报告。
刘易斯综合征是一种非常罕见的疾病。欧洲报道的首例病例（Lisch等人，2016年）在随访17年后从意义未明的双克隆丙种球蛋白病转变为MM。主观上，患者健康状况良好。对于眼科医生来说，Descemet膜水平典型的角膜盘状褐色混浊是铜代谢紊乱的可疑迹象。我们患者的铜水平极度升高。然而，在多次眼科检查中角膜混浊保持不变。2015年，我们这例刘易斯综合征患者转变为MM，通过不同阶段进行化疗和两次auto-SCT，导致MM完全缓解且血清铜水平正常。化疗后五年的观察期内双侧角膜混浊保持不变。
