PF183 POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME IN CHILDREN WITH HEMATOLOGHIC AND ONCOLOGIC DISEASES: MULTICENTER EXPERIENCE
Authors: Arman Bilir O. , Dikme G. , Malbora B. , Evim M.S. , OnderSivis Z. , Tufekci O. , Bahadır A. , Karaman S. , Vural S. , Bayhan T. , Celkan T. , Ozbek N.Y. 
Published: June 2019

Background:

Posterior reversible encephalopathy syndrome (PRES) is characterized with magnetic resonance imaging (MRI) findings involving hyper‐intense lesions in parieto‐occipital regions of cerebral cortex and subcortex accompanying with neurological manifestations. Incidence of PRES was reported to vary 1.6–3.95% in children with childhood leukemia. Neurological findings differs from generalized seizures to limb hypo‐aesthesia.

Aims:

The aim of this study was to demonstrate the characteristics’ of PRES cases reported from ten centers in Turkey.

Methods:

The demographic and clinical features of PRES cases reported from ten centers were assessed with a questionnaire. A retrospective analysis of the findings was performed.

Results:

A total of 56 patients (Female/Male 17/39) were analyzed. Mean ages were 8,16 ± 3,77 and 8,95 ± 3,66 years at initial diagnosis (respectively for underlying disease and PRES). Leukemia was the most common underlying disease (80.4%). PRES was diagnosed following stem cell transplantation in 5 patients whom all were receiving calcineurin inhibitors. PRES presented within 14 days following either chemotherapy (71,4%) or steroids (85.7%) regimen in patients with childhood malignancies. All were treated with BFM protocol and PRES occurred during induction in 26 (60%), consolidation in 8 (18%), and re‐induction in 10 (22%) patients. There was accompanying hypertension in 47 (83.9%) and infection in 26 (46.4%) patients, 22 of them (39.3%) had febrile neutropenia. The most common electrolyte disturbances were hypocalcemia (n = 9i 16.1%), hypomagnesemia (n = 8i 14.3%) and hypokalemia (n = 8, 14.3%). Tumor lysis syndrome occurred in 6 patients (10.7%) while syndrome of inappropriate antidiuretic hormone (SIADH) in 4 patients (7.1%). Lesions in MRI were found most commonly in occipital, parietal and frontal lobes respectively. Twenty five (44.6%) patients were admitted to PICU and 7 of them were intubated. Seizures, unconsciousness, visual impairment and headache were the main neurological manifestations. Thirty four of 45 patients received Phenytoin as anti‐epileptic therapy and 29 of them were under therapy for18 ± 24 months. Sixteen (28%) patients diagnosed as epilepsy. All of the patients received additional supporting therapies. One patient with PRES, sepsis and TLS died, others were fully recovered from PRES.

Summary/Conclusion:

We demonstrated that PRES occurred commonly in children with leukemia and mostly during induction therapy. We thought that steroids and multiple anti‐cancer drugs given during induction therapy was the primary cause of the PRES. Besides calcineurin inhibitors were the other risk factor for PRES in HSCT patients. However the exact drug causing PRES cannot be identified since patients were exposed to multiple drugs. Moreover many of the patients were also suffered from electrolyte imbalance or TLS. We diagnosed 4 SIADH cases which was rarely reported in the literature before. The prognosis in PRES patients were favorable with appropriate management and supportive therapy even the acute clinical picture is frightening and there were no re‐occurrence in any of the patients. Early detection of the findings, proper management and long‐term follow up is required and highly important