异基因造血干细胞移植导致的可逆性后部白质脑病综合征

Reversible posterior leukoencephalopathy syndrome caused by allogeneic hematopoietic stem cell transplantation

可逆性后部白质脑病综合征是异基因造血干细胞移植后常见并发症之一,病因主要是预处理方案中大剂量放化疗和免疫抑制剂的应用,另外与感染、抗感染治疗和移植后排斥反应有关。发病机制是脑血管自动调节障碍与内皮损伤、交感神经分布差异与高血压脑血管痉挛。临床表现多以抽搐为首发,多有视力下降、血压增高、头痛、意识和精神行为改变。全身症状可有代谢紊乱、低镁血症、低胆固醇血症、肾功能衰竭。可逆性后部白质脑病综合征一个重要特点是其临床和影像学的可逆性,异基因造血干细胞移植后仅少数患者在数周内痊愈,长时间抽搐、伴缺血性改变者预后不良。

Reversible posterior leukoencephalopathy syndrome is one of the common complications after allogeneic hematopoietic stem cell transplantation. The main etiologies of reversible posterior leukoencephalopathy syndrome are high-dose radiotherapy and chemotherapy in preparation, and the application of ~mmunosuppressive agents. Besides those, they may related to infections, anti-infection treatment and graft-versus-host disease. Its pathogenesis includes cerebrovascular autoregulation impaired and endothelial damage, differences in the distribution of sympathetic in cerebral vasospasm, as well as hypertension. Its first symptom is hyperspasmia, and the other clinical manifestations are hypopsia, hypertension, headache, awareness and mental behavioral changes. The general syndromes include metabolism disorder hypomagnesemia, low hypercholesterolemia, and renal function failure. The clinical manifestations and imaging are reversible, which is an important feature of reversible posterior leukoencephalopathy syndrome. A small number of patients were cured for several weeks after transplantation. Patient with tong seizure and ischemic change often have a poor prognosis.