Posterior Reversible Encephalopathy Syndrome after Allogeneic Stem Cell Transplantation in Pediatric Patients with Fanconi Anemia, a Prospective Study.

Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurologic complications following hematopoietic stem cell transplantation (HSCT). We aimed to evaluate the incidence, clinical, and imaging features of PRES in pediatric patients with Fanconi anemia (FA) following HSCT. This prospective study included all post-HSCT patients with underlying FA disease between 2014 and 2017. Brain computed tomography scan and magnetic resonance imaging (MRI) were performed in all individuals who developed neurologic symptoms. PRES was diagnosed based on clinic-radiological evidence. Follow-up MRI was performed in all patients with PRES within two months. Forty-one patients with FA (28 males; mean age, 8.19 ± 3.25 years) were enrolled. Out of 15 patients with acute neurologic symptoms, PRES was diagnosed in 9 individuals (21.95% of the total cohort). The occurrence of PRES was significantly higher in patients who had a donor with a 1-locus mismatch (P= .02). Donor relation, stem cell source, and graft-versus-host disease grade did not have any significant association with the development of PRES. MRI showed asymmetric vasogenic edema in 5 patients, an overt infarct in 1 patient, and foci of microhemorrhages in 3 patients, 1 of whom developed a hemorrhagic infarct. This patient died shortly, and persistent microhemorrhages were noted in the other 2 patients. Our findings demonstrate a greater risk of developing PRES after HSCT in patients with FA compared with those with other diseases (21.95% versus 1% to 10%), and in contrast to its term, it might be irreversible and has adverse effects on HSCT outcomes. The increased vascular and endothelial fragility in FA may contribute to the higher frequency of PRES in these individuals.

Clinical Effects of COVID-19 on Hematopoietic Stem Cell Transplant Outcomes in Pediatric Patients.

Coronavirus disease 2019 is the third zoonotic acute respiratory disease after SARS virus and Middle East respiratory syndrome. Most cases are mild in healthy children. In contrast, the infection is more severe in patients with underlying health conditions. Because there are few posttransplant reports in hematopoietic stem celltransplant patients, here we described COVID19 infection in 4 confirmed cases among pediatric hematopoietic stem cell transplant recipients: 3 boys and 1 girl with a median age of 6 years. Three patients presented with symptoms of lower respiratory tract disease, whereas 1 patient presented with extrapulmonary symptoms without fever or pulmonary involvement. All of the patients were on immunosuppressivedrugs, ie, 1patientforgraft-versus-hostdisease prophylaxis and 3 patients for graft-versus-host disease treatment.Thosewhowerediagnosedwith active graftversus-hostdisease requiredmechanical ventilationand intensive care. Two patients died from multiple organ dysfunction and resistant coinfection, and 1 patient developed pulmonary hypertension and mild cardiomegaly and remained at the hospital for more than 2 months, whereas the patient with no graft-versus-host disease was discharged and recovered. Our findings showed that COVID-19 infection among hematopoietic stem cell transplant recipients may be more severe and associatedwithlong-termhospitalization and complications. Active graft-versus-hostdisease, coinfections, and long-term use of immunosuppressive agents are risk factors for poor outcomes.