Management and Clinical Outcome of Posterior Reversible Encephalopathy Syndrome in Pediatric Oncologic/Hematologic Diseases: A PRES Subgroup Analysis With a Large Sample Size

Abstract

This study investigated the management and clinical outcomes along with associated factors of posterior reversible encephalopathy syndrome (PRES) in childhood hematologic/oncologic diseases. We present data from children with hematologic/oncologic diseases who developed PRES after treatment of the primary disease with chemotherapy and hematopoietic stem cell transplantation (HSCT) at 3 medical centers in Changsha, China from 2015 to 2020, and review all previously reported cases with the aim of determining whether this neurologic manifestation affects the disease prognosis. In the clinical cohort of 58 PRES patients, hypertension [pooled odds ratio (OR) = 4.941, 95% confidence interval (CI): 1.390, 17.570; P = 0.001] and blood transfusion (OR = 14.259, 95% CI: 3.273, 62.131; P = 0.001) were significantly associated with PRES. Elevated platelet (OR = 0.988, 95% CI: 0.982, 0.995; P < 0.001), hemoglobin (OR = 0.924, 95% CI: 0.890, 0.995; P < 0.001), and blood sodium (OR = 0.905, 95% CI: 0.860, 0.953; P < 0.001), potassium (OR = 0.599, 95% CI: 0.360, 0.995; P = 0.048), and magnesium (OR = 0.093, 95% CI: 0.016, 0.539; P = 0.008) were protective factors against PRES. Data for 440 pediatric PRES patients with hematologic/oncologic diseases in 21 articles retrieved from PubMed, Web of Science, and Embase databases and the 20 PRES patients from our study were analyzed. The median age at presentation was 7.9 years. The most common primary diagnosis was leukemia (62.3%), followed by solid tumor (7.7%) and lymphoma (7.5%). Most patients (65.0%) received chemotherapy, including non-induction (55.2%) and induction (44.8%) regimens; and 86.5% used corticosteroids before the onset of PRES. Although 21.0% of patients died during follow-up, in most cases (93.2%) this was not attributable to PRES but to severe infection (27.3%), underlying disease (26.1%), graft-vs.-host disease (14.8%), multiple organ dysfunction syndrome (8.0%), and respiratory failure (3.4%). PRES was more common with HSCT compared to chemotherapy and had a nearly 2 times higher mortality rate in patients with oncologic/hematologic diseases than in those with other types of disease. Monitoring neurologic signs and symptoms in the former group is therefore critical for ensuring good clinical outcomes following treatment of the primary malignancy.

Posterior reversible encephalopathy syndrome in immunocompromised children – A single-center study from South India

“…found that mortality among PRES with HSCT was two times that of oncologic or hematologic disease where 65% of patients were receiving chemotherapy of which 44.8% were in induction regimen and 86.5% were on corticosteroids before the onset of PRES. [ 6 ]…”

Complications of Cancer Therapy in Children: A Comprehensive Review of Neuroimaging Findings

“…Although it may develop in patients with many medical conditions (typically renal failure and hypertension), PRES can occur in children undergoing cancer treatment, most commonly in those with hematologic malignancies, and who have undergone allogeneic hematopoietic cell transplant. [8][9][10][11][12] Medications that have been associated with PRES include vincristine, cisplatin, methotrexate (MTX), L-asparaginase, and immunosuppressive agents. 13 Clinical symptoms may include seizures, altered mental status, and/or headache.…”

“…15 Treatment involves supportive care, antihypertensives, antiepileptics, and interruption of offending agent, if possible. 8,11,12 Although most clinical and imaging findings are transient, abnormalities on magnetic resonance imaging (MRI) and seizures may persist in some (particularly in the presence of abnormal diffusion-weighted imaging changes), and death may occur in rare cases. 8,9,11,12…”

“…Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic syndrome consisting of neurologic symptoms and neuroimaging findings. Although it may develop in patients with many medical conditions (typically renal failure and hypertension), PRES can occur in children undergoing cancer treatment, most commonly in those with hematologic malignancies, and who have undergone allogeneic hematopoietic cell transplant 8–12 . Medications that have been associated with PRES include vincristine, cisplatin, methotrexate (MTX), L-asparaginase, and immunosuppressive agents 13 .…”

Dental dilemmas in blood disorders: Navigating oral health in hematological diseases

“…Leukemia, particularly Acute Lymphoblastic Leukemia (ALL), a common pediatric cancer originating in the bone marrow, accounts for 20% of all childhood cancers. Despite treatment advancements, ALL remains a therapeutic challenge but boasts a promising prognosis, with a current 5-year overall survival rate of 90% [6] . It involves an excessive production of immature and abnormally differentiated leukocytes [7] .…”