关键词: autopsy myelodysplastic syndromes posterior reversible encephalopathy syndrome transfusion therapy

来  源:   DOI:10.1111/trf.17968

Abstract:
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is known as a transfusion-related complication with typically favorable prognosis and no report fatalities. Pathological evaluation of PRES is also scarce.
METHODS: An 88-year-old female with myelodysplastic syndromes (MDS) attended our hospital because of a compression fracture and chronic heart failure with chronic anemia. While her hemoglobin levels improved from 4.6 to 8.0 g/dL and the pleural effusions substantially decreased following six units of red blood cell transfusion and diuretic therapy, a gradual decline in cognitive function and speech reduction was noted. PRES was diagnosed by magnetic resonance imaging of the head. Despite treatment of intensive supportive care, the patient fell into a coma by the 20th day and passed away on the 22nd day. Although the pathophysiological link between blood-transfusion-related PRES and its impact on survival is not fully understood, autopsy findings confirmed the diagnosis of PRES and revealed multiple cerebral hemorrhages that were not detected in earlier imaging studies.
CONCLUSIONS: This case highlights the importance of vigilant monitoring and management of PRES, especially in high-risk populations such as elderly patients with multiple comorbidities or those with thrombocytopenia. Further studies are needed to elucidate the mechanisms of PRES in patients with hematologic diseases.
摘要:
背景:后部可逆性脑病综合征(PRES)是一种输血相关并发症,通常预后良好,无死亡报告。PRES的病理评估也很少。
方法:一名88岁的骨髓增生异常综合征(MDS)女性患者因压缩性骨折和慢性心力衰竭伴慢性贫血而到我院就诊。尽管她的血红蛋白水平从4.6g/dL提高到8.0g/dL,但经过6个单位的红细胞输注和利尿剂治疗后,胸腔积液明显减少,观察到认知功能和言语减少逐渐下降.PRES通过头部磁共振成像诊断。尽管接受了重症支持治疗,患者在第20天陷入昏迷,第22天去世。尽管与输血相关的PRES及其对生存的影响之间的病理生理联系尚未完全了解,尸检结果证实了PRES的诊断,并显示了早期影像学研究中未发现的多发性脑出血。
结论:此案例突出了警惕监测和管理PRES的重要性,特别是在高危人群中,如患有多种合并症的老年患者或血小板减少症患者。需要进一步的研究来阐明血液疾病患者PRES的机制。
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