The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of Systemic Lupus Erythematosus-Associated Hemophagocytic Lymphohistiocytosis Treated Successfully with an Etoposide Infusion

루푸스와 연관된 혈구포식 림프조직구증에서 Etoposide 병합치료 1예

  • Han, Sung Wan (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Kang, Kyu Keun (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Park, So Hyun (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Song, Ji Young (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Yu, Woong Sun (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Choi, Jung Ran (Department of Internal Medicine, Seoul Veterans Hospital) ;
  • Kim, Choon Kwan (Department of Internal Medicine, Seoul Veterans Hospital)
  • Received : 2012.05.14
  • Accepted : 2012.10.05
  • Published : 2013.04.01
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Abstract

Hemophagocytic lymphohistiocytosis (HLH) can develop following strong activation of the immune system and the cardinal symptoms are a prolonged fever, hematological abnormalities, hepatosplenomegaly, and hemophagocytosis. HLH can be classified as primary or secondary HLH, associated with infections, malignancy and autoimmune disease. There is no consensus on the primary treatment regimen in systemic lupus erythematosus (SLE)-associated HLH. We experienced a case of SLE-associated HLH in a previously healthy adult. She was initially treated with intravenous immunoglobulin, cyclosporine, and high-dose steroid, but had a poor clinical response. After intravenous etoposide, the patient stabilized and has been followed for 1 year without reactivation of the HLH or SLE.

50세 여자 환자를 SLE 연관 HLH로 진단하고, 면역억제치료에도 호전되지 않아 etoposide를 주사 후 호전된 증례 1예를 보고하는 바이다.

Keywords

References

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