自体造血干细胞移植治疗严重自身免疫性疾病

Z. Stojanoski, A. Karadzova-Stojanoska, Aleksandra Pivkova-Veljanovska, S. Genadieva-Stavrik, L. Cadievski, M. Ivanovski, O. Karanfilski, L. Cevreska, B. Georgievski
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摘要

摘要介绍。自身免疫性疾病是一个由100多种异质性疾病组成的家族,影响着世界人口的5%至8%。病因尚不清楚,但调节性t淋巴细胞的失调在自身免疫和长期缓解的成功中起着核心作用。虽然传统的免疫抑制和新的生物制剂可以为严重感染的患者提供疾病控制,但这些治疗方法很少能治愈,需要其他策略。事实上,严重的系统性自身免疫性疾病,如多发性硬化症(MS)、系统性硬化症(SSc)、类风湿性关节炎(RA)、系统性红斑狼疮(SLE)、幼年特发性关节炎(JIA)、血液学免疫性细胞减少症(HIC)和克罗恩病等,都很难治疗。高剂量免疫抑制治疗后自体干细胞移植是这组患者连续治疗的可靠选择。的目标。目的:探讨自体干细胞移植治疗自身免疫性疾病伴发恶性血液病的安全性。方法。在15年期间(2000年9月至2015年9月),我们在斯科普里大学血液学诊所治疗了6名患有自身免疫性疾病和伴随血液学肿瘤的患者。这些患者都没有接受过原发性自身免疫性疾病的治疗。2男4女,中位年龄47岁。干燥综合征和多发性骨髓瘤2例,结节性多动脉炎和多发性骨髓瘤1例,类风湿性关节炎和急性髓母细胞白血病1例,系统性红斑狼疮和非霍奇金淋巴瘤1例;严重银屑病合并急性髓母细胞白血病1例。结果。移植手术后所有接受治疗的患者均存活,移植相关死亡率为+100:0。结论。自体干细胞移植是安全的,是治疗自身免疫性疾病和血液肿瘤患者的推荐选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Treatment of Severe Autoimmune Diseases with Autologous Hematopoietic Stem Cell Transplantation
Abstract Introduction. Autoimmune diseases are a family of more than 100 heterogeneous conditions that affect 5 to 8% of the world’s population. The etiology is still un-known but the disregulation of the regulatory T-lymphocytes play a central role inthe autoimmunity and the success of the long-term remission. Although conventional immunosuppression and new biological agents can provide disease control in severely affected patients, such treatments are rarely curative and alternative strategies are needed. Indeed, severe forms of systemic autoimmune diseases, such as multiple sclerosis (MS), systemic sclerosis (SSc), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), juvenile idiopathic arthritis (JIA), hematologic immune cytopenia (HIC) and Crohn’s disease are difficult to be treated. High-dose immunosuppressive therapy followed by autologous stem cells transplantation is reliable option for a successive treatment of this group of patients. Aim. To determine the safety of the procedure of autologous stem cell transplantation in patients with autoimmune diseases and concomitant malignant hematological disorders. Methods. During a period of 15 years (from September 2000 to September 2015) at the University Clinic of Hematology in Skopje we have treated 6 patients with autoimmune disease and concomitant hematological neoplasm. None of the patients was treated for primary autoimmune diseases. Two men and 4 women, with median age of 47 years were treated. Sjogren syndrome and multiple myeloma were found in 2 patients, polyartheritis nodosa and multiple myeloma in 1 patient, rheumatoid arthritis and acute myeloblastic leukemia in 1, systemic lupus erythematosus and non-Hodgkin lymphoma in 1; severe psoriasis and acute myeloblastic leukemia in 1 patient. Results. All treated patients are alive after trans-planted procedure, with transplant related mortality day +100: 0. Conclusion. Autologous stem cell transplantation is safe and recommended option for treatment ofpatients with autoimmune disease and hematologic neoplasm.
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