异体移植治疗系统性红斑狼疮和严重再生障碍性贫血1例报告及简要文献回顾

Gomez-Almaguer D
{"title":"异体移植治疗系统性红斑狼疮和严重再生障碍性贫血1例报告及简要文献回顾","authors":"Gomez-Almaguer D","doi":"10.23880/mjccs-16000335","DOIUrl":null,"url":null,"abstract":"Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the bone marrow. Aplastic anemia (AA) is a rare but serious complication of this condition. Patients with AA secondary to SLE are usually treated with immunosuppression but less is known about hematopoietic stem cell transplantation (HSCT) as a therapeutic option. We present the case of a 22-year-old Latin-American woman with a known diagnosis of SLE referred to our center due to pancytopenia. She presented joint pain, fatigue, oral ulcers, a non-scarring alopecia, and anemic syndrome. A diagnostic workup revealed severe aplastic anemia (sAA). She received immunosuppressive therapy with corticosteroids, cyclosporine, danazol, and rituximab, which led to a transient partial response. An outpatient HLA-matched related allogeneic HSCT was performed. She received 5.9 x 106 CD34+ cells/kg after conditioning with high-dose cyclophosphamide plus fludarabine and anti-thymocyte globulin. She achieved a complete response and continues in remission with no signs of graft-versus-host disease (GVHD) or SLE activity at 38 months of follow-up. Even though allogeneic hematopoietic stem cell transplantation is not defined as a first-line treatment for severe AA in SLE, the procedure resulted in the complete remission of both related autoimmune diseases in this patient.","PeriodicalId":356296,"journal":{"name":"Medical Journal of Clinical Trials & Case Studies","volume":"19 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Curing Systemic Lupus Erythematosus and Severe Aplastic Anemia with an Allogeneic Transplant: A Case Report and Brief Literature Review\",\"authors\":\"Gomez-Almaguer D\",\"doi\":\"10.23880/mjccs-16000335\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the bone marrow. Aplastic anemia (AA) is a rare but serious complication of this condition. Patients with AA secondary to SLE are usually treated with immunosuppression but less is known about hematopoietic stem cell transplantation (HSCT) as a therapeutic option. We present the case of a 22-year-old Latin-American woman with a known diagnosis of SLE referred to our center due to pancytopenia. She presented joint pain, fatigue, oral ulcers, a non-scarring alopecia, and anemic syndrome. A diagnostic workup revealed severe aplastic anemia (sAA). She received immunosuppressive therapy with corticosteroids, cyclosporine, danazol, and rituximab, which led to a transient partial response. An outpatient HLA-matched related allogeneic HSCT was performed. She received 5.9 x 106 CD34+ cells/kg after conditioning with high-dose cyclophosphamide plus fludarabine and anti-thymocyte globulin. She achieved a complete response and continues in remission with no signs of graft-versus-host disease (GVHD) or SLE activity at 38 months of follow-up. Even though allogeneic hematopoietic stem cell transplantation is not defined as a first-line treatment for severe AA in SLE, the procedure resulted in the complete remission of both related autoimmune diseases in this patient.\",\"PeriodicalId\":356296,\"journal\":{\"name\":\"Medical Journal of Clinical Trials & Case Studies\",\"volume\":\"19 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1900-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Medical Journal of Clinical Trials & Case Studies\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.23880/mjccs-16000335\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Medical Journal of Clinical Trials & Case Studies","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.23880/mjccs-16000335","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

系统性红斑狼疮(SLE)是一种自身免疫性疾病,可影响许多器官,包括骨髓。再生障碍性贫血(AA)是一种罕见但严重的并发症。继发于SLE的AA患者通常采用免疫抑制治疗,但对造血干细胞移植(HSCT)作为一种治疗选择知之甚少。我们报告一位22岁的拉丁美洲女性,因全血细胞减少症而被诊断为SLE。她表现为关节痛、疲劳、口腔溃疡、非瘢痕性脱发和贫血综合征。诊断检查显示严重再生障碍性贫血(sAA)。她接受了皮质类固醇、环孢素、那那唑和利妥昔单抗的免疫抑制治疗,导致短暂的部分反应。门诊进行hla匹配相关同种异体造血干细胞移植。在大剂量环磷酰胺加氟达拉滨和抗胸腺细胞球蛋白治疗后,接受5.9 × 106个CD34+细胞/kg。在38个月的随访中,她获得了完全缓解并持续缓解,没有移植物抗宿主病(GVHD)或SLE活动的迹象。尽管同种异体造血干细胞移植未被定义为SLE严重AA的一线治疗方法,但该手术导致该患者两种相关自身免疫性疾病完全缓解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Curing Systemic Lupus Erythematosus and Severe Aplastic Anemia with an Allogeneic Transplant: A Case Report and Brief Literature Review
Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the bone marrow. Aplastic anemia (AA) is a rare but serious complication of this condition. Patients with AA secondary to SLE are usually treated with immunosuppression but less is known about hematopoietic stem cell transplantation (HSCT) as a therapeutic option. We present the case of a 22-year-old Latin-American woman with a known diagnosis of SLE referred to our center due to pancytopenia. She presented joint pain, fatigue, oral ulcers, a non-scarring alopecia, and anemic syndrome. A diagnostic workup revealed severe aplastic anemia (sAA). She received immunosuppressive therapy with corticosteroids, cyclosporine, danazol, and rituximab, which led to a transient partial response. An outpatient HLA-matched related allogeneic HSCT was performed. She received 5.9 x 106 CD34+ cells/kg after conditioning with high-dose cyclophosphamide plus fludarabine and anti-thymocyte globulin. She achieved a complete response and continues in remission with no signs of graft-versus-host disease (GVHD) or SLE activity at 38 months of follow-up. Even though allogeneic hematopoietic stem cell transplantation is not defined as a first-line treatment for severe AA in SLE, the procedure resulted in the complete remission of both related autoimmune diseases in this patient.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信