造血干细胞移植(HSCT)治疗慢性炎症性脱髓鞘多发性神经病(CIDP):是CIDP吗?

Q2 Medicine
Richard K Burt, Joachim Burman, Alexander Barker, Basil Sharrack
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引用次数: 0

摘要

约80%的慢性炎症性脱髓鞘多发性神经病(CIDP)患者在静脉注射免疫球蛋白和或血浆置换术失败后或对其产生依赖后,通过自体造血干细胞移植(HSCT)可获得5年无治疗缓解。自体造血干细胞移植可显著改善患者的力量、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振幅。造血干细胞移植的效果取决于对患者的正确选择,即正确的诊断和疾病的正确阶段。一个重要的注意事项是,许多被诊断为 CIDP 的患者在接受进一步检查后发现患有其他病因引起的周围神经病变。因 CIDP 而接受造血干细胞移植的患者应在造血干细胞移植前重新进行评估以确诊,造血干细胞移植失败的患者应重新进行评估以确诊 CIDP 以外的其他病因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hematopoietic stem cell transplantation (HSCT) for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): Is it CIDP?

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.

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来源期刊
Handbook of clinical neurology
Handbook of clinical neurology Medicine-Neurology (clinical)
CiteScore
4.10
自引率
0.00%
发文量
302
期刊介绍: The Handbook of Clinical Neurology (HCN) was originally conceived and edited by Pierre Vinken and George Bruyn as a prestigious, multivolume reference work that would cover all the disorders encountered by clinicians and researchers engaged in neurology and allied fields. The first series of the Handbook (Volumes 1-44) was published between 1968 and 1982 and was followed by a second series (Volumes 45-78), guided by the same editors, which concluded in 2002. By that time, the Handbook had come to represent one of the largest scientific works ever published. In 2002, Professors Michael J. Aminoff, François Boller, and Dick F. Swaab took on the responsibility of supervising the third (current) series, the first volumes of which published in 2003. They have designed this series to encompass both clinical neurology and also the basic and clinical neurosciences that are its underpinning. Given the enormity and complexity of the accumulating literature, it is almost impossible to keep abreast of developments in the field, thus providing the raison d''être for the series. The series will thus appeal to clinicians and investigators alike, providing to each an added dimension. Now, more than 140 volumes after it began, the Handbook of Clinical Neurology series has an unparalleled reputation for providing the latest information on fundamental research on the operation of the nervous system in health and disease, comprehensive clinical information on neurological and related disorders, and up-to-date treatment protocols.
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