Korean J Med.  2013 Apr;84(4):613-618.

A Case of Systemic Lupus Erythematosus-Associated Hemophagocytic Lymphohistiocytosis Treated Successfully with an Etoposide Infusion

Affiliations
  • 1Department of Internal Medicine, Seoul Veterans Hospital, Seoul, Korea. schenics@naver.com

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.

Keyword

Lymphohistiocytosis; Hemophagocytic; Etoposide; Systemic; Lupus Erythematosus

MeSH Terms

Adult
Autoimmune Diseases
Consensus
Cyclosporine
Etoposide
Fever
Humans
Immune System
Immunoglobulins
Lupus Erythematosus, Systemic
Lymphohistiocytosis, Hemophagocytic
Cyclosporine
Etoposide
Immunoglobulins
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