■后部可逆性脑病综合征(PRES)是一种严重的神经系统综合征,可能在干细胞移植(SCT)的免疫抑制治疗后发展。我们报告了8例镰状细胞病(SCD)患者发展为PRES,这可能与免疫抑制有关。
■这是利雅得国王费萨尔专科医院和研究中心(KFSHRC)的SCD注册的回顾性队列分析,沙特阿拉伯。纳入标准包括2011年至2022年接受SCT的所有成人SCD患者。我们探讨了SCT患者的所有PRES病例。PRES被诊断为MRI成像显示可逆性血管源性脑水肿与神经症状相关,包括严重头痛,癫痫发作,脑病,谵妄,和视觉障碍。
■在10年的随访(2011-2022年)中,我们发现8例PRES患者(诊断时年龄在14至37岁之间)在SCT后8至124天发生PRES,其中7例,1例患者在SCT前8个月发生PRES。所有患者都在服用免疫抑制药物,包括他克莫司,环孢菌素,西罗莫司和或霉酚酸酯。头痛,癫痫发作,视觉幻觉,混乱,嗜睡是最常见的症状。MRI显示枕骨异常,顶叶和额叶在大多数情况下。所有患者均完全恢复,无复发。两名患者患有移植物抗宿主病(GVHD)。我们比较了未发生PRES的SCD患者8例和136例SCT中PRES的危险因素。PRES与影像学异常之间存在显着关联,包括以前的双半球梗死(p=0.001),和脑微出血(CBMs)。PRES与存在密切相关(p=0.006),CMB的大小(p=0.016)和数量(p=0.005)。
■在SCD患者中,PRES可以在SCT后几天到几周发展,与免疫抑制治疗有关,先前的双半球梗塞和CMB。及时的识别和干预导致良好的恢复。
UNASSIGNED: Posterior reversible encephalopathy syndrome (PRES) is a serious neurological syndrome that may develop following immunosuppressive therapy for stem cell transplantation (SCT). We report 8 patients with sickle cell disease (SCD) who developed PRES, which is likely to be related to immunosuppression.
UNASSIGNED: This is retrospective cohort analysis of the SCD registry at the King Faisal Specialist Hospital and Research Center (KFSHRC) in Riyadh, Saudi Arabia. Inclusion criteria included all adults SCD patients who underwent SCT from 2011 until 2022. We explored all cases of PRES in patients with SCT. PRES was diagnosed with MRI imaging showing reversible vasogenic cerebral edema associated with neurological symptoms including severe headache, seizures, encephalopathy, delirium, and visual disturbances.
UNASSIGNED: During ten years follow-up (2011-2022) we found 8 patients with PRES (age range between 14 to 37 years at diagnosis) PRES occurred 8 to 124 days following SCT in 7 cases and one patient developed PRES 8 months prior to SCT. All patients were on immunosuppressive medications, including tacrolimus, cyclosporine, sirolimus and or mycophenolate mofetil. Headache, seizures, visual hallucinations, confusion, and drowsiness were the most common presenting symptoms. MRI showed abnormalities in the occipital, parietal and frontal lobes in most cases. Recovery was complete in all patients and no recurrences were noted. Two patients had graft versus host disease (GVHD). We compared risk factors for PRES among the 8 cases and 136 SCT in SCD patients who did not develop PRES. There was a significant association between PRES and imaging abnormalities, including previous bi-hemispheric infarctions (p = 0.001), and cerebral microbleeds (CBMs). PRES was strongly associated with presence (p = 0.006), size (p = 0.016) and number (p = 0.005) of CMBs.
UNASSIGNED: PRES can develop days to weeks following SCT in patients with SCD, and is associated with immunosuppressive therapy, previous bi-hemispheric infarctions and CMB. Prompt recognition and intervention leads to good recovery.