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Early diagnosis and successful treatment of paraneoplastic melanocytic proliferation
  1. Joyce C G Jansen1,
  2. Joachim Van Calster1,
  3. Jose S Pulido2,
  4. Sarah L Miles3,
  5. Richard G Vile4,
  6. Tine Van Bergen5,
  7. Catherine Cassiman1,
  8. Leigh H Spielberg6,
  9. Anita M Leys1
  1. 1Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium
  2. 2Departments of Molecular Medicine and Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Biochemistry and Microbiology, Marshall University, Huntington, West Virginia, USA
  4. 4Department of Immunology and Molecular Medicine Mayo Clinic, Rochester, Minnesota, USA
  5. 5Department of Neuroscience, University Hospitals Leuven, Leuven, Belgium
  6. 6Department of Ophthalmology Rotterdam Eye Hospital, Rotterdam, The Netherlands
  1. Correspondence to Dr Joyce C G Jansen, Department of Ophthalmology, University Hospitals Leuven, 33 Kapucijnenvoer, Leuven 3000, Belgium; joyce.jansen{at}uzleuven.be

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.

  • Retina
  • Imaging
  • Choroid
  • Neoplasia

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