Jansen Joyce C G, Van Calster Joachim, Pulido Jose S, Miles Sarah L, Vile Richard G, Van Bergen Tine, Cassiman Catherine, Spielberg Leigh H, Leys Anita M
Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
Departments of Molecular Medicine and Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
Br J Ophthalmol. 2015 Jul;99(7):943-8. doi: 10.1136/bjophthalmol-2014-305893. Epub 2015 Apr 23.
Paraneoplastic melanocytic proliferation (bilateral diffuse uveal melanocytic proliferation, BDUMP) is a rare but devastating disease that causes progressive visual loss in patients who usually have an occult malignancy. Visual loss occurs as a result of paraneoplastic changes in the uveal tissue.
In a masked fashion, the serum of two patients with BDUMP was evaluated for the presence of cultured melanocyte elongation and proliferation (CMEP) factor using cultured human melanocytes. We evaluated the efficacy of plasmapheresis as a treatment modality early in the disease in conjunction with radiation and chemotherapy.
The serum of the first case patient was investigated after plasmapheresis and did not demonstrate proliferation of cultured human melanocytes. The serum of the second case was evaluated prior to treatment with plasmapheresis and did induce this proliferation. These findings are in accordance with the diminution of CMEP factor after plasmapheresis. Treatment with plasmapheresis managed to stabilise the ocular disease progression in both patients.
In the past, visual loss due to paraneoplastic melanocytic proliferation was considered progressive and irreversible. We treated two patients successfully with plasmapheresis and demonstrated a relation between CMEP factor in the serum of these patients and proliferation of cultured melanocytes.
副肿瘤性黑素细胞增殖（双侧弥漫性葡萄膜黑素细胞增殖，BDUMP）是一种罕见但具有破坏性的疾病，通常会导致患有隐匿性恶性肿瘤的患者视力逐渐丧失。视力丧失是葡萄膜组织发生副肿瘤性改变的结果。
采用盲法，使用培养的人黑素细胞评估两名BDUMP患者血清中培养的黑素细胞伸长和增殖（CMEP）因子的存在情况。我们在疾病早期联合放疗和化疗评估了血浆置换作为一种治疗方式的疗效。
第一例患者在血浆置换后对其血清进行了检测，未显示培养的人黑素细胞增殖。第二例患者在进行血浆置换治疗前对其血清进行了评估，确实诱导了这种增殖。这些发现与血浆置换后CMEP因子的减少一致。血浆置换治疗成功稳定了两名患者的眼部疾病进展。
过去，副肿瘤性黑素细胞增殖导致的视力丧失被认为是进行性且不可逆的。我们成功地用血浆置换治疗了两名患者，并证明了这些患者血清中的CMEP因子与培养的黑素细胞增殖之间的关系。
