Departments of Ophthalmology, American University of Beirut and Rafic Hariri University Hospital, Beirut, Lebanon.
Retina Departments, The King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia, and Wilmer Eye Institute, The Johns Hopkins University, Baltimore, Maryland.
Ophthalmology. 2014 Oct;121(10):1925-32. doi: 10.1016/j.ophtha.2014.04.007. Epub 2014 Jun 18.
Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits.
Multicenter, retrospective, observational case series.
The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed.
Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings.
Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies.
A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity.
Paraproteinemic maculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.
单克隆丙种球蛋白血症与产生病理性抗体有关，浆液性黄斑脱离是一种罕见的眼部表现。我们确定了副蛋白血症患者的黄斑病变的临床病程，并研究了各种治疗方法对视网膜下沉积物消退的影响。
多中心、回顾性、观察性病例系列。
对光学相干断层扫描（OCT）记录有浆液性黄斑脱离的副蛋白血症患者的病历进行了回顾。
数据收集包括并存疾病、流变学数据（免疫球蛋白水平、红细胞压积和血液粘度）、临床检查结果和 OCT 发现。
最佳矫正视力（BCVA）、浆液性黄斑脱离的高度和基底面积以及全身与局部治疗。
共收集了 33 例病例：10 例为新病例，23 例为文献报道的病例。7 例患者有糖尿病，9 例患者有高血压，18 例患者有贫血。初始免疫球蛋白水平平均为 6497mg/dl，血清粘度平均为 5.5 厘泊（cP）。右眼初始与最终 BCVA 的最小角分辨率对数分别为 0.55（Snellen 等效值，20/71）和 0.45（20/56），左眼分别为 0.38（20/48）和 0.50（20/63）。在平均 7 个月（0-51 个月）的随访后。全身治疗包括血浆置换（18 例）、化疗（30 例）、输血（2 例）、造血祖细胞移植（2 例）和口服利妥昔单抗（10 例）。8 例患者的免疫球蛋白水平正常，1 例患者在接受血浆置换、化疗或两者联合治疗后未改变。8 例患者的眼部治疗包括玻璃体切除术（1 例）、激光光凝（4 例）、玻璃体内贝伐单抗（5 例）、玻璃体内曲安奈德（2 例）、玻璃体内地塞米松植入物（1 例）、玻璃体内利妥昔单抗（1 例）和 Tenon 下皮质类固醇（1 例）。17 例患者的黄斑病变部分或完全缓解，14 例患者的黄斑病变恶化或保持不变，中位随访时间为 7 个月。9 例为单侧病变，糖尿病患者的初始免疫球蛋白水平较低。脱离面积与血清粘度呈正相关。
副蛋白血症性黄斑病变可单侧发生。降低血液免疫球蛋白水平是治疗副蛋白血症性黄斑病变的主要目标，可通过全身途径实现。并存的糖尿病可促进免疫球蛋白在低于非糖尿病患者的水平漏出。
