Bilateral Marginal Keratitis Associated With Engraftment Syndrome After Hematopoietic Stem Cell Transplantation

Abstract

Purpose:: To describe the first case of bilateral marginal keratitis in the setting of engraftment syndrome in a patient status post hematopoietic stem cell transplantation.

Methods:: Case report and literature review.

Results:: A 63 year old man with a history of myelodysplastic syndrome presented with a five day history of red eyes to the Ophthalmology clinic at MD Anderson Cancer Center. Two weeks prior to presentation, the patient had received a matched unrelated donor peripheral blood stem cell transplant after reduced intensity chemotherapy regimen. The patient developed engraftment syndrome with a rapid white blood cell recovery of 0.2 x10³ cells/µl to 9x10³ cells/µl in one day, fever, rash, and shortness of breath which quickly resolved without increase in oral corticosteroids. Blood cultures and chest X-ray were negative for infection, and there was no evidence of hyperacute graft versus host disease (GVHD). Ocular symptoms coincided with the timing of the white blood cell recovery. On examination, the vision was 20/20 OU with +1 conjunctival injection with bilateral corneal subepithelial infiltrate 360 degrees around the cornea, particularly in the areas of the corneal pannus from his previously existing contact lens related neovascularization. There was no evidence of blepharitis, meibomian gland dysfunction, follicles, papillae or discharge, and no evidence of uveitis in the anterior or posterior chamber. The patient had normal Schirmer's test and the rest of the examination was unremarkable. Ocular bacterial and viral cultures were negative and a conjunctival biopsy was negative for viral inclusions or ocular GVHD. The patient was initially treated with topical antibiotics for 2 days which did not improve his condition and was then treated with topical corticosteroids four times daily. Symptoms resolved in 4 days and examination showed resolution of the marginal keratitis in 7 days. No ocular manifestations of engraftment syndrome have been previously described in the literature.

Conclusions:: We report the first case of bilateral marginal keratitis associated with engraftment syndrome. Engraftment syndrome is notable for a rapid recovery of the white blood cell count after hematopoietic stem cell transplantation. Patients who present with bilateral conjunctivitis in the setting of autologous and allogeneic stem cell transplantation should be evaluated for engraftment syndrome related marginal keratitis.

Keywords: cornea: epithelium • keratitis