耐火材料(行星科学)
重症肌无力
医学
胸腺切除术
破伤风
环磷酰胺
免疫系统
免疫疗法
免疫学
白喉
类毒素
血浆置换术
白喉毒素
抗体
内科学
胃肠病学
化疗
接种疫苗
生物
生物化学
天体生物学
毒素
作者
Daniel B. Drachman,Robert N. Adams,Rong Hu,Richard J. Jones,Robert A. Brodsky
标识
DOI:10.1196/annals.1405.033
摘要
A small but important proportion of patients with myasthenia gravis (MG) are refractory to conventional immunotherapy. We have treated 12 such patients by “rebooting” the immune system with high‐dose cyclophosphamide (Hi Cy, 200 mg/kg), which largely eliminates the mature immune system, while leaving hematopoietic precursors intact. The objective of this report is to describe the clinical and immunologic results of Hi Cy treatment of refractory MG. We have followed 12 patients clinically for 1–9 years, and have analyzed their humoral and cellular immunologic parameters. Hi Cy is safe and effective. All but one of the patients experienced dramatic clinical improvement for variable periods from 5 months to 7.5 years, lasting for more than 1 year in seven of the patients. Two patients are still in treatment‐free remission at 5.5 and 7.5 years, and five have achieved responsiveness to immunosuppressive agents that were previously ineffective. Hi Cy typically reduced, but did not completely eliminate, antibodies to the autoantigen AChR or to tetanus or diphtheria toxin; re‐immunization with tetanus or diphtheria toxoid increased the antibody levels. Despite prior thymectomy, T cell receptor excision circles, generally considered to reflect thymic emigrant T cells, were produced by all patients. Hi Cy treatment results in effective, but often not permanent, remission in most refractory myasthenic patients, suggesting that the immune system is in fact “rebooted,” but not “reformatted.” We therefore recommend that treatment of refractory MG with Hi Cy be followed with maintenance immunotherapy.
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