From the Department of Pediatric Hematology-Oncology and Bone Marrow Transplantation, Acibadem Adana Hospital, Adana, Turkey.
Exp Clin Transplant. 2024 Oct;22(10):800-809. doi: 10.6002/ect.2024.0126.
Hematopoietic stem cell transplant is increasingly being used as a curative therapeutic option for patients with hematologic malignancies and nonmalignant diseases. Here, we aimed to determine the frequency and features of typical and atypical central variants of posterior reversible encephalopathy syndrome in children who had undergone an allogeneic hematopoietic stem cell transplant procedure for any indication at a single center.
We retrospectively analyzed 101 pediatric patients between aged 6 months and 18 years who had undergone hematopoietic stem cell transplant for any indication between 2010 and 2023. We evaluated morbidity and mortality rates and several risk factors, therapies, and outcomes associated with posterior reversible encephalopathy syndrome. Medical data were obtained from patients' electronic health records.
In 101 patients who underwent hematopoietic stem cell transplant, the most common diagnosis was acute lymphoblastic leukemia (n = 46; 45.5%). Median time to onset of posterior reversible encephalopathy syndrome was 20 days (range, 10-108 days) posttransplant. Most cases of posterior reversible encephalopathy syndrome were typical, and 16 patients (15.8%) had the central variant. Headache was the most common clinical manifestation (79.2%). Rates of steroid use and complete remission in patients with the central variant were significantly (P < .05) lower than in the group with the typical variant. Mortality rates were significantly higher in patients with the central variant (n = 10, 62.5%) compared with those with the typical variant (n = 5, 5.9%) (P < .001).
Patients with the central variant of posterior reversible encephalopathy syndrome showed significantly increased risk of mortality. Because this clinical-radiological condition frequently manifests with atypical findings on magnetic resonance imaging, consideration of posterior rever-sible encephalopathy syndrome as a potential diagnosis is crucial when the clinical presentation is consistent but radiological findings are not typical to posterior reversible encephalopathy syndrome.
造血干细胞移植越来越多地被用作治疗血液系统恶性肿瘤和非恶性疾病患者的方法。在此，我们旨在确定在单个中心接受同种异体造血干细胞移植治疗任何疾病的儿童中，典型和非典型后部可逆性脑病综合征中枢变体的频率和特征。
我们回顾性分析了 2010 年至 2023 年间因任何原因接受造血干细胞移植的 101 名 6 个月至 18 岁的儿科患者。我们评估了后部可逆性脑病综合征的发病率、死亡率以及与后部可逆性脑病综合征相关的若干风险因素、治疗方法和结局。从患者的电子健康记录中获取医学数据。
在 101 名接受造血干细胞移植的患者中，最常见的诊断是急性淋巴细胞白血病（n = 46；45.5%）。移植后出现后部可逆性脑病综合征的中位时间为 20 天（范围，10-108 天）。大多数后部可逆性脑病综合征病例为典型，16 例（15.8%）为中枢变体。头痛是最常见的临床表现（79.2%）。中枢变体组患者使用类固醇和完全缓解的比例明显（P <.05）低于典型变体组。中枢变体组患者的死亡率明显高于典型变体组（n = 10，62.5%）与典型变体组（n = 5，5.9%）（P <.001）。
后部可逆性脑病综合征中枢变体患者的死亡率显著增加。由于这种临床-放射学情况在磁共振成像上经常表现出非典型表现，因此当临床表现一致但放射学表现不典型时，应考虑将后部可逆性脑病综合征作为潜在的诊断。
