Vol.22 No.2

Case Report

Etanercept for the treatment of intractable hemophagocytic syndrome with systemic lupus erythematosus

Authors

Hirotoshi Kikuchi1,2 , Tadashi Yamamoto2 , Kurumi Asako2 , Maki Takayama2 , Ryosuke Shirasaki2 , Yasuo Ono1,2

  • Department of Microbiology and Immunology, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan
  • Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, 173-8605, Japan
Received:

8 April 2011

Accepted:

27 June 2011

Published online:

12 July 2011

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Abstract

A 41-year-old woman presented with continuous fever, and her laboratory data suggested the recrudescence of systemic lupus erythematosus. She was treated with 60 mg/day prednisolone. With a dose reduction of prednisolone, high fever and pancytopenia were observed again. A bone marrow biopsy revealed hemophagocytosis. The effects of steroid pulse therapy, high-dose intravenous immunoglobulin, cyclosporine A, and methotrexate were insufficient. However, after four injections of etanercept (25 mg, twice a week) subcutaneously, her symptoms had completely resolved. In such cases, therapy with etanercept may be effective.

Key words

Systemic lupus erythematosus - Hemophagocytic syndrome - Etanercept - CD64 - Procalcitonin