CD34 SELECTED PERIPHERAL BLOOD PROGENITOR CELLS W/ IMMUNOBLATION

严重的自身免疫性疾病只能通过常规的免疫抑制治疗得到部分控制。 这些疾病有40多种，包括严重的狼疮、混合性结缔组织病和多发性硬化症。 一般来说，这些疾病被认为是慢性疾病，但其中一小部分是侵袭性的，可迅速导致死亡。 免疫抑制药物，特别是皮质类固醇，一直是治疗的支柱，细胞毒性药物被用于更难治疗的病例。 异常的免疫细胞将自我识别为外来的东西，会被排斥，这是导致这些疾病相关问题的原因。 这项研究建议使用高剂量化疗，放射治疗和移植去除免疫细胞的造血干细胞的组合，以消除那些对自身产生反应的免疫细胞，并用一种不识别自身为外来物的新免疫系统取代它们。 这些手术的风险主要包括与高剂量化疗和放疗相关的风险，严重器官损伤的风险较低，手术相关死亡的移植几率高达5%。 为了确保只有疾病处于适当阶段的患者才能接受此程序，所有转诊必须由杜兰医疗中心的适当学术人员进行审查，包括风湿病学，神经病学，儿科免疫学和其他适当的团体。此外，必要时将请外部专家审查可能的候选人。 Tulane骨髓移植计划中适用于癌症患者的所有要求，包括患者生理和心理功能，将适用于本研究的患者。向患者提供的替代治疗将包括进一步使用标准疗法。 然而，这些患者将被认为是标准治疗或其他研究性治疗失败的患者，因为这些特定疾病实体可能变得可用。

Severe autoimmune diseases can only partially be controlled by conventional immunosuppressive treatments. There are over 40 of these diseases including severe lupus, mixed connective tissue disease and multiple sclerosis. In general, these diseases have been considered chronic diseases, but a small percentage of them are aggressive and can lead rapidly to death. Immunosuppressive drugs, particularly corticosteroids, have been the mainstay of therapy, with cytotoxic agents being used in the more difficult to treat cases. Abnormal immune cells, which recognize self as something foreign to be rejected, are responsible for causing the problems related to these diseases. This study proposes to use a combination of high dose chemotherapy, with radiation therapy and the transplantation of blood stem cells depleted of immune cells in order to eliminate those immune cells that react to self, and replace them with a new immune system that does not recognize self as foreign. The risks of these procedures include predominantly those associated with high dose chemotherapy and radiation therapy, with lower risk of severe organ damage, and up to a 5 % chance of transplant of procedure related death. In order to assure that only patients with the appropriate stages of their diseases are referred for this procedure, all referrals must be reviewed by the appropriate academic faculty at Tulane Medical Center including rheumatology, neurology, pediatric immunology and other appropriate groups. In addition, where necessary, outside experts will be called upon to review potential candidates. All requirement of the Tulane Bone Marrow Transplant Program that apply to cancer patients, regarding patients physiologic and psychological functioning, will be applied to patients on this study. Alternative treatments to be offered to the patients will include further use of standard therapies. However, these will be patients who have been felt to have failed standard therapy or other investigational treatments as may become available for those specific disease entities.