Most children diagnosed with acute leukemia are cured without hematopoietic stem cell transplantation (HSCT), but for some high-risk subgroups and in relapsed disease, allogeneic HSCT can significantly enhance the probability of relapse-free survival. 1 Cytomegalovirus (CMV) infection is of minor clinical significance in immunocompetent hosts, but occurs frequently in patients with impaired cellular immunity including allogeneic HSCT recipients. 2 Despite major advances in management, recurrent CMV infection and disease remain the most important viral cause for morbidity and mortality in patients post HSCT. Recurrent CMV infection is defined as new CMV infection in patients with previous evidence of CMV infection but no virus detection for an interval of at least 4 weeks during active surveillance. It may result from either reactivation of latent virus (endogenous) or reinfection (exogenous). 3The occurrence of CMV detection in blood in pediatric patients undergoing allogeneic HSCT for leukemia has been estimated at up to 43%. 4 Ocular manifestations of CMV disease in the form of CMV retinitis (CMVR) are more rarely reported in the general population of HSCT recipients (0.2%-8%), but, relative to adults, appear to be more frequent in children. 5-10 For acute leukemia, the exact
AbstractCytomegalovirus retinitis (CMVR) may occur after allogeneic hematopoietic stem cell transplantation (HSCT). However, little is known about its incidence, strategies for ophthalmic surveillance, and timely implementation of adequate antiviral treatment in pediatric allogeneic HSCT recipients. We provide a retrospective analysis of the epidemiology and clinical features of CMVR in pediatric allogeneic HSCT patients transplanted at our center over a 16-year period. Two patients of this cohort with leukemia are presented. Our analysis is supplemented by a systematic review on pediatric patients with leukemia and CMVR in the setting of allogeneic HSCT. The overall incidence of CMVR in our cohort was 1% (4/338) and 14.2% (3/21) in leukemic patients. In published cases, CMVR occurred at a median of 143 days after transplantation, and, in the majority of patients, was preceded by CMV detection in blood by a median of 93 days. Continued immune suppression following engraftment likely triggers CMVR. Preemptive treatment with ganciclovir as standard is usually successful. Foscarnet is used in case of resistance to ganciclovir or drug-induced granulocytopenia. Overall, CMVR after HSCT in pediatric leukemic patients is rare, but a potentially higher vulnerability of this population for involvement of the eye warrants a standardized ophthalmological examination plan.
K E Y W O R D Schildren, cytomegalovirus, leukemia, retinitis, transplantation