Case Report The Potential Role of Plasma Exchange as a Treatment for Bilateral Diffuse Uveal Melanocytic Proliferation: A Report of Two Cases Elizabeth A. Jaben, 1 Jose S. Pulido, 2 Sean Pittock, 3 Svetomir Markovic, 4 and Jeffrey L. Winters 1 * 1 Department of Laboratory Medicine and Pathology, Division of Transfusion Medicine, Mayo Clinic, Rochester, MN 2 Department of Ophthalmology, Mayo Clinic, Rochester, MN 3 Department of Neurology, Mayo Clinic, Rochester, MN 4 Division of Hematology, Mayo Clinic, Rochester, MN 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. Pathologi- cally, 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. J. Clin. Apheresis 26:356–361, 2011. V V C 2011 Wiley Periodicals, Inc. Key words: bilateral diffuse uveal melanocytic proliferation; plasma exchange; paraneoplastic syndrome INTRODUCTION Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome with only 31 patients suffering from this disorder having been reported in the medical literature [1–25]. The disorder presents with rapid-onset vision loss in patients in their 50s through 80s. The ocular findings and vision loss often appear before recognition of the underlying malignancy [1–4,7–10,12,15,16,21,22], but may become apparent after diagnosis of the malignancy in up to half of cases [3–6,8–10,13,17–19,23–25]. BDUMP is most commonly associated with a gynecologic carci- noma in women [8–10,12,17–19,21,23,24] and lung [2,3,4,15,16,25] or pancreatic [1,3,10,22] cancer in men. Clinically, BDUMP is characterized by five ophthal- mologic findings: multiple round, red patches in the posterior fundus; multifocal hyperfluorescence corre- sponding to the patches; slightly elevated, pigmented, and nonpigmented uveal melanocytic tumors and diffuse thickening of the uveal tract; serous retinal detachment; and rapidly developing cataracts [22]. Surgical removal of cataracts often improves vision in patients, but not to the extent that would be anticipated. Histologically, diffuse uveal tract thickening is the most common finding [22]. Additionally, pigmentation of the skin and mucosal membranes may be present in these patients representing proliferation of melanocytes in areas other than the uveal tract [7,13,21]. Treatment options for patients with BDUMP are very limited and rapid blindness occurs. In the past, bilateral enucleation of the eyes was performed due to concern that the melanocytic proliferation represented bilateral uveal melanoma [22]. In the cases reported in the literature to date, vision loss occurs in nearly all cases, and may occur before or after diagnosis of a primary malignancy (Table I). Cancer-associated Abbreviations used: BDUMP, bilateral diffuse uveal melanocytic proliferation; PE, plasma exchange; CAR, cancer-associated retinop- athy; FFP, fresh frozen plasma. *Correspondence to: Jeffrey L. Winters, Division of Transfusion Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905. E-mail: winters.jeffrey@mayo.edu Received 17 January 2011; Accepted 15 July 2011 Published online 5 September 2011 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jca.20310 V V C 2011 Wiley Periodicals, Inc. Journal of Clinical Apheresis 26:356–361 (2011)