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异基因造血干细胞移植治疗白塞病合并骨髓增生异常综合征:一例报告及文献复习

Application of allogeneic hematopoietic stem cell transplantation to treat Behcet's disease with myelodysplastic syndrome: a case report and literature review.

作者信息

Lei RuiQi, Liu Xin, Wu YiTing, Song JingYu, Lv XiaoLi, Liu Jie, Guo Dan, Li HuiBo, Fan ShengJin

机构信息

Division of Hematology, Department of Medicine, The First Affiliated Hospital of Harbin Medical University, 23 Youzheng Street, Nan Gang District, Harbin, 150001, Heilongjiang Province, China.

NHC Key Laboratory of Cell Transplantation, the First Affiliated Hospital, Harbin Medical University, Harbin, 150001, China.

出版信息

Ann Hematol. 2024 Dec;103(12):6015-6020. doi: 10.1007/s00277-024-06122-y. Epub 2024 Nov 29.

Abstract

Trisomy 8 is a frequent chromosomal abnormality that occurs among patients with myelodysplastic syndrome (MDS). This abnormality is more common among MDS patients with concurrent Behcet's disease (BD) than among patients with MDS alone. This comorbidity is also associated with a higher prevalence of intestinal ulcers. In this case report, we describe a 45-year-old woman who was diagnosed with BD at age 35. Eight years later, her Behcet's disease relapsed with trisomy 8-positive MDS. After the disease was controlled by azacitidine, the patient received allogeneic hematopoietic stem cell transplantation (allo-HSCT), which involved the use of both peripheral blood stem cells and bone marrow stem cells. Although the patient experienced grave oral mucositis and chronic intestinal graft-versus-host disease (GVHD), remission of both MDS and BD was maintained. This case highlights the potential efficacy of allo-HSCT in treating patients with refractory autoimmune diseases complicated by MDS, particularly patients with trisomy 8 abnormalities. We also discuss the immunological implications and suggest allo-HSCT as a viable curative option for patients with BD and MDS.

摘要

8号染色体三体是骨髓增生异常综合征(MDS)患者中常见的染色体异常。这种异常在并发白塞病(BD)的MDS患者中比仅患有MDS的患者更为常见。这种合并症还与肠道溃疡的较高患病率相关。在本病例报告中,我们描述了一名45岁女性,她在35岁时被诊断为BD。八年后,她的白塞病复发并伴有8号染色体三体阳性的MDS。在疾病通过阿扎胞苷得到控制后,患者接受了异基因造血干细胞移植(allo-HSCT),该移植使用了外周血干细胞和骨髓干细胞。尽管患者经历了严重的口腔黏膜炎和慢性肠道移植物抗宿主病(GVHD),但MDS和BD均维持缓解。该病例突出了allo-HSCT在治疗并发MDS的难治性自身免疫性疾病患者,特别是具有8号染色体三体异常患者中的潜在疗效。我们还讨论了免疫学意义,并建议allo-HSCT作为BD和MDS患者可行的治愈选择。

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