title: Early diagnosis and successful treatment of paraneoplastic melanocytic proliferation.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Joyce C G
authors.identifier: http://orcid.org/0000-0002-9300-4195
authors.initials: JC
authors.lastname: Jansen
authors.affiliation: Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Joachim
authors.initials: J
authors.lastname: Van Calster
authors.affiliation: Departments of Molecular Medicine and Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Jose S
authors.initials: JS
authors.lastname: Pulido
authors.affiliation: Department of Biochemistry and Microbiology, Marshall University, Huntington, West Virginia, USA.
authors.forename: Sarah L
authors.initials: SL
authors.lastname: Miles
authors.affiliation: Department of Immunology and Molecular Medicine Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Richard G
authors.initials: RG
authors.lastname: Vile
authors.affiliation: Department of Neuroscience, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Tine
authors.initials: T
authors.lastname: Van Bergen
authors.affiliation: Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Catherine
authors.initials: C
authors.lastname: Cassiman
authors.affiliation: Department of Ophthalmology Rotterdam Eye Hospital, Rotterdam, The Netherlands.
authors.forename: Leigh H
authors.initials: LH
authors.lastname: Spielberg
authors.affiliation: Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Anita M
authors.initials: AM
authors.lastname: Leys
mesh_terms: D000230:Adenocarcinoma / Q000556:secondary* / Q000628:therapy; D000368:Aged; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000068258:Bevacizumab; D049109:Cell Proliferation / Q000187:drug effects; D003131:Combined Modality Therapy; D042241:Early Diagnosis; D005451:Fluorescein Angiography; D006801:Humans; D058449:Intravitreal Injections; D008175:Lung Neoplasms / Q000473:pathology* / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008544:Melanocytes / Q000473:pathology*; D059545:Paraneoplastic Syndromes, Ocular / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D010956:Plasmapheresis; D058471:Subretinal Fluid; D041623:Tomography, Optical Coherence; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors; D014792:Visual Acuity / Q000502:physiology; D058609:Visual Field Tests; D014794:Visual Fields
chemical_list: D020533:Angiogenesis Inhibitors; D061067:Antibodies, Monoclonal, Humanized; D000970:Antineoplastic Agents; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab
keywords: Choroid; Imaging; Neoplasia; Retina