ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2019, Vol. 28 ›› Issue (3): 293-297.DOI: 10.3969/j.issn.1006-298X.2019.03.021

• 论文 • 上一篇    下一篇

系统性红斑狼疮合并噬血细胞综合征

  

  • 出版日期:2019-06-28 发布日期:2019-07-23

Lupus erythematosus complicated by hemophagocytic syndrome

  • Online:2019-06-28 Published:2019-07-23

摘要:

18岁女性患者,以腹痛、发热起病,进而出现肝酶升高及全血细胞减少、癫痫发作等,腹部CT提示假性肠梗阻,骨髓细胞学检查有噬血细胞,血清学检查抗核抗体阳性,EB病毒感染再激活。最终确诊为系统性红斑狼疮、EB病毒感染,并发假性肠梗阻和噬血细胞综合征。

关键词: 系统性红斑狼疮, 噬血细胞综合征, 巨噬细胞活化综合征, 假性肠梗阻

Abstract:

An 18year old female patient,began with abdominal pain,and fever,then developed pancytopenia, transaminase elevation and seizureComputed tomography(CT) revealed intestinal pseudoobstruction(IPO) and bone marrow aspirate proved hemophagocytes.Serologic tests for EpsteinBarr virus(EBV) indicated its reactivation and antinuclear antibodies positive.This case demonstrates that hemophagocytic  syndrome (HPS)  and concomitant IPO associated with EBV reactivation can occur as clinical manifestations of systemic lupus erythematosus(SLE) flare.

Key words: systemic lupus erythematosus, hemophagocytic syndrome, macrophage activation syndrome, intestinal pseudoobstruction