Curing Systemic Lupus Erythematosus and Severe Aplastic Anemia with an Allogeneic Transplant: A Case Report and Brief Literature Review

Gomez-Almaguer D
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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.
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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
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。
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