Pediatr Blood Cancer 2015;62:721–723 BRIEF REPORT Red Cell Exchange Transfusion Halts Progressive Proliferative Sickle Cell Retinopathy in a Teenaged Patient With Hemoglobin SC Disease Christopher M. McKinney, MD, 1 Frank Siringo, MD,OD, 2 Jeffrey L. Olson, MD, 2 Kelly E. Capocelli, MD, 3 Daniel R. Ambruso, MD, 1,3 and Rachelle Nuss, MD 1,4 * INTRODUCTION Sickle cell disease encompasses a family of inherited hemoglobinopathies causing chronic hemolysis and small vessel vaso-occlusion. The double heterozygous variant, hemoglobin SC, is associated with an increased frequency of ophthalmic compli- cations including proliferative sickle cell retinopathy (PSCR). PSCR usually presents during the third decade of life and affects 43% of patients with sickle SC disease [1]. However, approximately 8–20% of pediatric patients with sickle SC disease will develop PSCR during childhood [2]. PSCR is staged according to the Goldberg Classification System [3]. Stage I disease indicates peripheral arterial occlusion. In Stage II, peripheral arteriovenous anastomoses (hairpin loops) are present. Stage III disease is characterized by intraretinal neovas- cularization and fibrous proliferation. The presence of vitreous hemorrhage defines Stage IV disease. Without treatment, the most advanced disease results in retinal detachment (Stage V). The goal of treatment for PSCR is to prevent vision loss by preventing recurrent vitreous hemorrhage, retinal detachment and epiretinal membrane development. Five to ten percent of untreated individuals suffer vision loss [4]. However, treatment of PSCR is stage dependent and controversial, although photocoagulation is generally considered the first-line treatment for Stage III or greater PSCR [5]. Case Description At 8 years of age, a male with sickle SC disease presented with Stage III PSCR which spontaneously resolved. His prior sickle cell complications included two hospitalizations for acute chest syndrome (ACS) and three for vaso-occlusive crises (VOC). From 8–13 years of age he required hospitalization on four occasions, all for VOC, one in association with ACS. At 13 years of age, he complained of a “black line in vision of the left eye” lasting 5 days. At that presentation his best-corrected visual acuity was 20/25 and 20/25 in the right and left eyes, respectively. A dilated fundoscopic exam of the right eye revealed large areas of peripheral retinal non-perfusion with bilateral active and regressed neovascularization inferotemporally and a small vitreous hemorrhage in his left eye. His baseline hemoglobin was 14 g/dl with a hemoglobin electrophoresis consistent with hemoglobin SC disease showing 3.9% A2, 0.4% F, 44.4% C and 51.3% S hemoglobin; the diagnosis was confirmed by DNA analysis of his beta globin chains. Examination under anesthesia of the right eye demonstrated a partially auto-regressed sea fan temporally, a small area of pre-retinal fibrosis along the posterior border of the superotemporal/nasal vasculature. In the left eye (Fig. 1), exam demonstrated PSCR, with a partially auto-infarcted sea fan peripherally with vitreous hemor- rhage on the inferior edge, preretinal hemorrhage, and areas of fibrosis inferiorly, superiorly and supranasally. Sector panretinal photocoagulation was performed using an Argon laser with 351 spots and 1700 spots of 0.1 sec pulses and 220 milliWatt power to the right temporal sea fan and left sea fan/ ischemic retina, respectively. Despite repeated treatment with laser photocoagulation, the patient developed progressive PSCR over the following 30 months with recurrent vitreous hemorrhages in both retinas as shown in Figure 2. His vision ranged from 20/25 to 20/60 in the right eye and 20/25 to 20/400 in the left eye. During that interval, he also required hospitalization in the intensive care unit for ACS treated with exchange transfusion and inhaled nitric oxide and two hospitalizations for VOC. The teenager underwent a pars plana vitrectomy of the right eye to clear the vitreous hemorrhage and facilitate further laser retinal photocoagulation. Due to his ongoing visual compromise despite A male with sickle SC disease presented at age 8 years with proliferative sickle cell retinopathy (PSCR) and bilateral vitreous hemorrhage which spontaneously resolved, then recurred at 13 years of age. Despite conventional therapy with repeated pan-retinal photocoagulation and pars plana vitrectomy, he developed progres- sive PSCR and recurrent vitreous hemorrhage over the next 30 months. We describe the successful use of chronic red cell exchange transfusion (RCE) to preserve his vision and stabilize the retinopathy. Pediatr Blood Cancer 2015;62:721–723. # 2015 Wiley Periodicals, Inc. Key words: hemoglobin SC disease; red cell exchange; retinopathy; sickle cell Abbreviations: PSCR, Proliferative Sickle Cell Retinopathy; RCE, Red Cell Exchange; VOC, Vaso-occlusive Crisis; ACS, Acute Chest Syndrome; RBC, Red Blood Cells 1 Department of Pediatrics, Center for Cancer and Blood Disorders, Children’s Hospital Colorado, University of Colorado Denver, Aurora, Colorado; 2 Department of Ophthalmology, University of Colorado Denver, Aurora, Colorado; 3 Department of Pathology, Children’s Hospital Colorado, University of Colorado Denver, Aurora, Colorado; 4 Sickle Cell Treatment and Research Center, Children’s Hospital Colorado, University of Colorado Denver, Aurora, Colorado Conflict of interest: Nothing to declare.  Corresponding to: Rachelle Nuss, 13123 E 16 th Ave, B115, Aurora, CO 80045. E-mail: rachelle.nuss@childrenscolorado.org Received 13 October 2014; Accepted 19 November 2014  C 2015 Wiley Periodicals, Inc. DOI 10.1002/pbc.25397 Published online 28 January 2015 in Wiley Online Library (wileyonlinelibrary.com).