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慢性移植物抗宿主病相关肌肉痉挛:严重程度及对免疫调节治疗的反应

Chronic Graft-versus-Host Disease-Associated Muscle Cramps: Severity and Response to Immunomodulatory Therapies.

作者信息

Rastogi Supriya, Kim Ellen J, Gelfand Joel M, Loren Alison W, Baumrin Emily

机构信息

Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.

Division of Hematology/Oncology, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

出版信息

Transplant Cell Ther. 2024 Mar;30(3):338.e1-338.e6. doi: 10.1016/j.jtct.2023.12.674. Epub 2024 Jan 3.

Abstract

Muscle cramps in patients with chronic graft-versus-host disease (cGVHD) are common and associated with impaired quality of life and symptom burden. Muscle cramps are not currently captured in the 2014 National Institutes of Health (NIH) response criteria, and thus characterization and response to immunomodulatory therapies are lacking. The objective of this study was to characterize muscle cramp frequency, duration, and pain level in patients with steroid-refractory cGVHD undergoing extracorporeal photopheresis (ECP). A single-center cohort of patients who underwent ECP for the indication of steroid-refractory cGVHD with muscle cramps at treatment initiation were followed from April 2021 to April 2023. Of 22 patients receiving ECP for cGVHD during the study period, 9 (41%) had muscle cramps at ECP initiation (6 males [66%]; median age, 59 years; range, 25 to 66 years). Seven of these 9 patients (78%) had multiple organs involved, and 7 (78%) had severe disease by the NIH Global Severity scale. Over a median treatment duration of 28 weeks (range, 10 to 48 weeks), 8 patients (89%) had decreased frequency of muscle cramps from a median of 5 episodes per week (range, 3 per day to 2 per week) to a median of <1 episode per week (range, 1 per month to 3 per week). The pain and duration of muscle cramps were not changed meaningfully. The NIH Global Severity score remained unchanged in 6 patients (67%) and was improved in 3 patients (33%). Muscle cramping is a morbid feature of cGVHD that may be sensitive to change with standard immunomodulatory therapies. Muscle cramp frequency should be further validated as a response measure in cGVHD.

摘要

慢性移植物抗宿主病(cGVHD)患者的肌肉痉挛很常见,且与生活质量受损和症状负担相关。目前2014年美国国立卫生研究院(NIH)的反应标准未纳入肌肉痉挛情况,因此缺乏对免疫调节治疗的特征描述及反应情况。本研究的目的是描述接受体外光化学疗法(ECP)的类固醇难治性cGVHD患者的肌肉痉挛频率、持续时间和疼痛程度。对2021年4月至2023年4月期间因类固醇难治性cGVHD伴肌肉痉挛而接受ECP治疗的单中心队列患者进行了随访。在研究期间接受ECP治疗的22例cGVHD患者中,9例(41%)在开始ECP治疗时有肌肉痉挛(6例男性[66%];中位年龄59岁;范围25至66岁)。这9例患者中有7例(78%)涉及多个器官,7例(78%)根据NIH全球严重程度量表患有严重疾病。在中位治疗持续时间28周(范围10至48周)内,8例患者(89%)的肌肉痉挛频率从每周中位数5次发作(范围从每天3次到每周2次)降至每周中位数<1次发作(范围从每月1次到每周3次)。肌肉痉挛的疼痛和持续时间没有明显变化。6例患者(67%)的NIH全球严重程度评分保持不变,3例患者(33%)有所改善。肌肉痉挛是cGVHD的一种病态特征,可能对标准免疫调节治疗的变化敏感。肌肉痉挛频率应作为cGVHD反应指标进一步验证。

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