Peking University People's Hospital, Peking University Institute of Hematology, No. 11Xizhimen South Street, Beijing, Xicheng District, China.
Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing, China.
Ann Hematol. 2018 Aug;97(8):1399-1406. doi: 10.1007/s00277-018-3299-4. Epub 2018 Mar 22.
Haploidentical hematopoietic stem cell transplant (haplo-HSCT) is an upfront and effective therapy for hematology patients, but it usually has many complications, such as neurological complications. As one of the neurological complications following haplo-HSCT, immune-mediated demyelinating diseases of the central nervous system (CNS) seriously affect a patient's quality of life. However, the incidence, risk factors, and pathogenesis of CNS demyelination are not very well understood. Thirty of the 1526 patients (1.96%) suffered from CNS demyelination. In univariate analysis, we found that blood-brain barrier (BBB) permeability and the CSF IgG synthesis index (IgG-Syn) were related to the occurrence of CNS demyelination (p < 0.05). In a multivariate analysis, the IgG-Syn (OR = 1.017, 95% CI 1.003-1.031, p = 0.019) and CSF anti-myelin oligodendrocyte glycoprotein antibody (MOG.Ab) (OR = 12.059, 95% CI 1.141-127.458, p = 0.038) were independently associated with the onset of CNS demyelination. We also studied the possible pathogenesis of CNS demyelination. Immune reconstitution (the cell proportions of CD19 B cells, CD3 T cells, and CD4 T cells); the counts of leucocytes, lymphocytes, monocytes, and platelets; and the levels of immunoglobulins A, G, and M 30, 60, and 90 days after HSCT showed no significant differences between CNS demyelination and no demyelination (p > 0.05). The probabilities of overall survival showed no significant differences between patients with and without demyelination (p > 0.05). Only four deaths in 30 patients, but bringing projected survival to less than 20%.We imply that IgG-Syn and CSF MOG. Ab may be associated with the onset of CNS demyelination during 2 weeks of neurological symptoms in patients with brain or spinal cord MRI abnormality. Immune reconstitution may not be the pathogenesis of CNS demyelination.
单倍体造血干细胞移植(haplo-HSCT)是血液学患者的一种直接有效的治疗方法,但它通常有许多并发症,如神经系统并发症。作为 haplo-HSCT 后的神经系统并发症之一,中枢神经系统(CNS)免疫介导的脱髓鞘疾病严重影响患者的生活质量。然而,CNS 脱髓鞘的发病率、危险因素和发病机制尚不清楚。1526 例患者中有 30 例(1.96%)发生 CNS 脱髓鞘。在单因素分析中,我们发现血脑屏障(BBB)通透性和 CSF IgG 合成指数(IgG-Syn)与 CNS 脱髓鞘的发生有关(p<0.05)。在多因素分析中,IgG-Syn(OR=1.017,95%CI 1.003-1.031,p=0.019)和 CSF 抗髓鞘少突胶质细胞糖蛋白抗体(MOG.Ab)(OR=12.059,95%CI 1.141-127.458,p=0.038)与 CNS 脱髓鞘的发病独立相关。我们还研究了 CNS 脱髓鞘的可能发病机制。免疫重建(CD19 B 细胞、CD3 T 细胞和 CD4 T 细胞的细胞比例);白细胞、淋巴细胞、单核细胞和血小板计数;以及 IgG、IgG 和 IgM 在 HSCT 后 30、60 和 90 天的水平在 CNS 脱髓鞘和无脱髓鞘之间没有显著差异(p>0.05)。有和无脱髓鞘患者的总生存率无显著差异(p>0.05)。30 例患者中有 4 例死亡,但预计生存率低于 20%。我们暗示 IgG-Syn 和 CSF MOG.Ab 可能与脑或脊髓 MRI 异常患者出现神经系统症状的 2 周内 CNS 脱髓鞘的发生有关。免疫重建可能不是 CNS 脱髓鞘的发病机制。