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重症肌无力的全身照射:长期随访

Total body irradiation for myasthenia gravis: a long-term follow-up.

作者信息

Durelli L, Ferrio M F, Urgesi A, Poccardi G, Ferrero B, Bergamini L

机构信息

Clinica delle Malattie del Sistema Nervoso, Università di Torino, Turin, Italy.

出版信息

Neurology. 1993 Nov;43(11):2215-21. doi: 10.1212/wnl.43.11.2215.

DOI:10.1212/wnl.43.11.2215
PMID:8232932
Abstract

Total body irradiation (TBI) produces prolonged immunosuppression with rare side effects. We studied 12 thymectomized patients affected with chronic generalized severe myasthenia gravis. All patients had been totally or partially refractory to prolonged oral treatment with immunosuppressive drugs, and most had contra-indications for these drugs. Low-dose (1.8- to 2.3-Gy total dose) TBI was administered in single, 0.1-Gy doses, two to three times per week. TBI was well tolerated and was associated with objective clinical improvement in six patients, lasting more than 2 years in five. In addition, TBI produced a long-lasting lymphopenia with a pronounced decrease of T CD4+ lymphocytes; T CD8+ lymphocytes were almost unchanged over the 2 years of the study. CD16+ and CD20+ lymphocytes, after an initial decrease, increased above baseline. TBI was also associated with decreased anti-AChR antibody titer. The decrease of lymphocyte count and of anti-AChR antibody titer was more pronounced in the patients who improved, suggesting that lymphopenia and immunosuppression may have contributed to clinical improvement.

摘要

全身照射(TBI)会产生持久的免疫抑制,且副作用罕见。我们研究了12例患有慢性全身性重症肌无力的胸腺切除患者。所有患者长期口服免疫抑制药物均完全或部分无效,且大多数患者对这些药物存在禁忌证。低剂量(总剂量1.8至2.3戈瑞)TBI以单次0.1戈瑞剂量、每周两至三次的方式进行给药。TBI耐受性良好,6例患者出现客观的临床改善,其中5例改善持续超过2年。此外,TBI导致持久的淋巴细胞减少,T CD4 + 淋巴细胞显著减少;在研究的2年中,T CD8 + 淋巴细胞几乎没有变化。CD16 + 和CD20 + 淋巴细胞在最初减少后,增加至基线水平以上。TBI还与抗AChR抗体滴度降低有关。淋巴细胞计数和抗AChR抗体滴度的降低在病情改善的患者中更为明显,这表明淋巴细胞减少和免疫抑制可能有助于临床改善。

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