Department of Medicine, Scripps Health Care, La Jolla, CA, United States; Genani Corporation, Chicago, IL, United States.
Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
Handb Clin Neurol. 2024;202:199-238. doi: 10.1016/B978-0-323-90242-7.00002-X.
Autologous hematopoietic stem cell transplantation (HSCT) is associated with 5-year treatment-free remissions in approximately 80% of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed or were dependent on intravenous immunoglobulin and or plasmapheresis. Autologous HSCT was associated with significant improvement in strength, independent ambulation, quality of life, nerve conduction velocity, and compound muscle action potential amplitude. The results of HSCT are dependent on proper patient selection, i.e., the right diagnosis and the right stage of the disease. An important caveat is that a significant number of patients with a CIDP diagnostic label are found upon further workup have a peripheral neuropathy of another etiology. Patients undergoing HSCT for CIDP should be reevaluated before HSCT to confirm the diagnosis and those who fail HSCT should be reevaluated for a diagnosis other than CIDP.
自体造血干细胞移植(HSCT)可使约 80%的慢性炎症性脱髓鞘性多发性神经病(CIDP)患者在失败或依赖静脉免疫球蛋白和/或血浆置换的情况下获得 5 年无治疗缓解。自体 HSCT 可显著改善肌力、独立行走能力、生活质量、神经传导速度和复合肌肉动作电位幅度。HSCT 的结果取决于患者的正确选择,即正确的诊断和疾病的正确阶段。一个重要的注意事项是,经过进一步检查,发现相当数量的CIDP 诊断标签患者患有另一种病因的周围神经病。接受 HSCT 治疗 CIDP 的患者应在 HSCT 前重新评估以确认诊断,而 HSCT 失败的患者应重新评估除 CIDP 以外的其他诊断。