Red cell exchange transfusion halts progressive proliferative sickle cell retinopathy in a teenaged patient with hemoglobin SC disease

Pediatr Blood Cancer. 2015 Apr;62(4):721-3. doi: 10.1002/pbc.25397. Epub 2015 Jan 28.

Abstract

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 progressive 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.

Keywords: hemoglobin SC disease; red cell exchange; retinopathy; sickle cell.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Child
  • Erythrocyte Transfusion*
  • Hemoglobin SC Disease / complications
  • Hemoglobin SC Disease / therapy*
  • Humans
  • Male
  • Retinal Diseases / etiology
  • Retinal Diseases / therapy*
  • Vitreous Hemorrhage / etiology
  • Vitreous Hemorrhage / therapy*