Posterior Reversible Encephalopathy Syndrome Associated with Cyclosporine Use in a Child Undergoing Allogeneic Hematopoietic Stem Cells Transplantation for Fanconi Anemia: A Case Report

Posterior reversible encephalopathy syndrome (PRES) is a neuroclinical and radiological syndrome that commonly consists of parietooccipital and posterior frontal cortical and subcortical edema. The neurologic manifestations are variable, but frequently include headache, altered mental status, visual disturbances and seizures. Known associated causative agents include calcineurin inhibitor immunosuppressives such as tacrolimus and cyclosporine.

 We herein report a case of 12 years old boy that was diagnosed with fanconi anemia and underwent allogeneic Hematopoietic Stem Cells Transplantation (HSCT) with Fludarabine (35 mg/m²/day x 5), Cyclophosphamide (10 mg/m²/day x4) and Anti-thymocyte Globulin (Rabbit ATG) (2.5 mg/kg/day x4).  GVHD prophylaxis included Cyclosporine (65 mg/m²/dose BID) starting day -3 and Mycophenolate Mofetil 600 mg/ m^2/dose BID) starting on day 0. Pre stem cells infusion course was complicated with ICU admission due to ATG anaphylaxis reaction. Post stem cells infusion, the patient had elevated blood pressure and experienced seizure.  MRI brain confirmed PRES. Cyclosporine was discontinued.  Patient was put on methylprednisolone 2 mg/kg for GVHD prophylaxis. The patient did not experience any seizure afterwards. PRES resolved based on follow up imaging. The patient was started on tacrolimus with no further PRES episodes. 

Although it is a rare complication, it can be concluded that PRES should be suspected with neurological symptoms in children undergoing HSCT and taking a calcineurin inhibitor. If confirmed by imaging, rigorous control of arterial blood pressure and discontinuation of the offending agent is recommended. We also conclude that it’s safe to rechallenge the patient with a different calcineurin inhibitor once the PRES resolves.