OPHTHALMIC SURGERY, LASERS & IMAGING 1 ■ CASE REPORT ■ Ocular Findings in a Patient With Castleman’s Disease Before and After Treatment With Immunosuppression and Plasmapheresis Rodrigo Jorge, MD, PhD Ingrid U. Scott, MD, MPH Rafael C. Oliveira, MD, PhD Rogério A. Costa, MD, PhD Rubens C. Siqueira, MD, PhD Paulo Louzada-Júnior, MD, PhD ABSTRACT A 12-year-old girl with a 3-month history of epistaxis and Castleman’s disease presented with blurred vi- sion in both eyes for 2 weeks. Indirect ophthalmos- copy revealed a blurred optic disc margin, venous engorgement and tortuosity, intraretinal hemorrhages and cotton wool spots, and serous detachment of the neurosensory retina in the posterior pole of each eye. Fluorescein angiography and laboratory tests revealed abnormalities consistent with the clinical examination. Six months following institution of immunosuppres- sive treatment, cryoglobulin levels decreased and visual acuity and funduscopic abnormalities were markedly improved. However, a few microaneurysms, retinal hemorrhages, and venous engorgement and tortuosity persisted. One month after the cessation of immuno- suppressive treatment, symptoms related to the hyper- viscosity syndrome recurred and the patient was treated with one session of plasmapheresis. One month after the plasmapheresis, the patient’s symptoms resolved, laboratory values were normal, visual acuity was 20/15 in both eyes, and the funduscopic examination of each eye was unremarkable. [Ophthalmic Surg Lasers Im- aging 2010;41:e1-e4.] INTRODUCTION Castleman’s disease, also called angiofollicular lymph node hyperplasia, is a rare atypical lymphopro- liferative disorder characterized by massive growth of lymphoid tissue. It has two clinical forms: the localized form and the multicentric form. The diagnosis is based on clinical findings supported by pathological features and the disease may be associated with several auto- immune features, including cryoglobulinemia, posi- tive antinuclear antibodies, and hypergammaglobu- linemia. 1 Cryoglobulinemias are characterized by elevated concentrations of cryoglobulins in the serum and may lead to hyperviscosity syndrome. Cryoglobulins are im- munoglobulins or immunoglobulin-containing com- plexes that precipitate spontaneously and form a gel at low temperatures. They become soluble again when the temperature rises. There are three major types of cryoglobulins. Type I is a single monoclonal immu- noglobulin with only one class or subclass of heavy or light chain that is related to lymphoproliferative dis- eases and could cause hyperviscosity syndrome. 2 Type From the Retina and Vitreous Service–Department of Ophthalmology, Otorhinolaryngology and Head and Neck Surgery (RJ, RCO, RAC, RCS), Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil; the Departments of Ophthalmology and Public Health Sciences (IUS), Penn State College of Medicine, Hershey, Pennsylvania; the Division of Clinical Immunology–Department of Internal Medicine (PL-J), Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil; and the Department of Medicine (RCO), Federal University of Rondônia, Porto Velho, Rondônia, Brazil. Originally submitted June 26, 2010. Accepted for publication July 29, 2010. Posted online September 29, 2010. The authors have no financial or proprietary interest in the materials presented herein. Address correspondence to Rafael C. Oliveira, MD, PhD, School of Medicine, Federal University of Rondônia, Av. Calama, Porto Velho, Rondônia, Brazil, 2264 Cep: 78900-000. E-mail: rafaeloftalmo@uol.com.br doi: 10.3928/15428877-20100929-10