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病例报告:小儿系统性红斑狼疮罕见再生障碍性贫血:病例系列及系统文献综述

Case Report: Rare aplastic anemia in pediatric systemic lupus erythematosus: a case series and systematic literature review.

作者信息

Gao Xianfei, Li Ying, Lan Xuerong, Zeng Ping

机构信息

Department of Pediatric, Allergy Immune and Rheumatology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health, Guangzhou, China.

出版信息

Front Pediatr. 2025 Jun 27;13:1602651. doi: 10.3389/fped.2025.1602651. eCollection 2025.

Abstract

BACKGROUND

Systemic lupus erythematosus (SLE) is a complex autoimmune disease in which the immune system mistakenly attacks healthy cells and tissues. This condition can impact various organs and systems, including the hematological system, where aplastic anemia (AA) emerges as a particularly severe and rare complication. Relevant clinical manifestations and treatment experiences must be shared and updated urgently.

METHODS

This manuscript presents the clinical features, examination, and treatment of two pediatric SLE patients with AA. And a systemic search of MEDLINE, EMBASE, LILACS, SciELO, and Scopus, using MeSH headings and keywords for "Aplastic Anaemia" and "Systemic Lupus Erythematosus".

RESULTS

Both of our cases were severe, resulting in one death. Our systematic literature review identified 32 eligible studies. We found a total of 38 patients, and along with the two patients from our case series, there were 9 out of 40 SLE patients who experienced alopecia areata as children. In all 40 patients, 52% (21 out of 40) developed AA at the onset of SLE. The infection rate was concerning, with 16 out of 40 patients affected. While 83% (33 out of 40) of the patients showed improved outcomes, all seven patients who died had contracted an infection.

CONCLUSION

SLE with AA is rare in both children and adults. Although the prognosis of this dangerous disease is optimistic in terms of data, it is still necessary to be vigilant, and early bone marrow examination, infection prevention, and on-time treatment are crucial.

摘要

背景

系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,免疫系统会错误地攻击健康细胞和组织。这种疾病会影响包括血液系统在内的多个器官和系统,其中再生障碍性贫血(AA)是一种特别严重且罕见的并发症。必须紧急分享和更新相关的临床表现及治疗经验。

方法

本文介绍了两名患有再生障碍性贫血的小儿SLE患者的临床特征、检查及治疗情况。并通过使用医学主题词(MeSH)以及“再生障碍性贫血”和“系统性红斑狼疮”的关键词,对MEDLINE、EMBASE、LILACS、SciELO和Scopus进行了全面检索。

结果

我们的两个病例都很严重,导致一人死亡。我们的系统文献综述确定了32项符合条件的研究。我们共发现38例患者,连同我们病例系列中的两名患者,40例SLE患者中有9例在儿童时期出现斑秃。在所有40例患者中,52%(40例中的21例)在SLE发病时出现再生障碍性贫血。感染率令人担忧,40例患者中有16例受到影响。虽然83%(40例中的33例)的患者预后有所改善,但所有7例死亡患者均感染。

结论

伴有再生障碍性贫血的SLE在儿童和成人中均很罕见。尽管就数据而言这种危险疾病的预后较为乐观,但仍需保持警惕,早期骨髓检查、预防感染和及时治疗至关重要。

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本文引用的文献

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Herpes Simplex Virus-Associated Aplastic Anemia.单纯疱疹病毒相关性再生障碍性贫血
Cureus. 2023 Feb 22;15(2):e35320. doi: 10.7759/cureus.35320. eCollection 2023 Feb.
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[Guidelines for the diagnosis and management of aplastic anemia in China (2022)].《中国再生障碍性贫血诊断与治疗指南(2022年版)》
Zhonghua Xue Ye Xue Za Zhi. 2022 Nov 14;43(11):881-888. doi: 10.3760/cma.j.issn.0253-2727.2022.11.001.
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Hepatitis-Associated Aplastic Anemia.肝炎相关性再生障碍性贫血。
Hematol Oncol Stem Cell Ther. 2022 Jun 1;15(2):8-12. doi: 10.1016/j.hemonc.2020.10.001.

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