The potential role of plasma exchange as a treatment for bilateral diffuse uveal melanocytic proliferation: a report of two cases

J Clin Apher. 2011 Dec;26(6):356-61. doi: 10.1002/jca.20310. Epub 2011 Sep 5.

Abstract

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome associated with gynecologic malignancies in women and pancreatic or lung carcinomas in men. The clinical presentation consists of the rapid onset of decreased visual acuity due to bilateral serous retinal detachment and cataracts. Pathologically, there is diffuse uveal thickening and proliferation of uveal melanocytes. The onset of blindness is often rapid, with some patients presenting with blindness. We describe the cases of two women with gynecologic malignancies who were treated with plasma exchange (PE) for BDUMP. After a course of five to seven procedures, their ocular disease stabilized. One patient has maintained her vision more than 1 year following the completion of the course of PE. The other patient, who also received treatment with corticosteroids, in addition to the PE, reported stable vision on telephone follow-up 9 months after presentation. These cases suggest that PE may be a treatment option in this rare paraneoplastic syndrome which has otherwise been reported to invariably result in vision loss.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Female
  • Genital Neoplasms, Female / complications
  • Humans
  • Male
  • Paraneoplastic Syndromes, Ocular / diagnosis
  • Paraneoplastic Syndromes, Ocular / etiology
  • Paraneoplastic Syndromes, Ocular / therapy*
  • Plasma Exchange* / methods
  • Visual Acuity

Substances

  • Adrenal Cortex Hormones