Vol.22 No.2

Case Report

Oral low-dose tacrolimus therapy for refractory hemophagocytic syndrome associated with systemic lupus erythematosus

Authors

Hideyuki Watanabe1 , Nobuhisa Hirase1 , Hideaki Goda1 , Hiroshi Nishikawa1 , Shoichiro Ikuyama1

  • Department of Rheumatology, Hematology, and Metabolic Diseases, Kyushu University Hospital at Beppu, 4546 Tsurumibaru, Beppu, Oita, 874-0838, Japan
Received:

25 January 2011

Accepted:

3 June 2011

Published online:

1 July 2011

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Abstract

Hemophagocytic syndrome (HPS) is an unusual disorder associated with systemic lupus erythematosus (SLE). A 64-year-old woman was admitted because of fever and urticarial vasculitis. Laboratory data revealed pancytopenia and immunological abnormalities, suggesting elevated disease activity. Prednisolone monotherapy failed to improve the pancytopenia despite the amelioration of other clinical findings. Because her condition was suggestive of HPS, tacrolimus at 2-3 mg/day was added to the prednisolone regimen. Eventually, the pancytopenia improved and prednisolone could be effectively tapered. Tacrolimus could be an additional or alternative modality for treating refractory HPS.

Key words

Hemophagocytic syndrome - Systemic lupus erythematosus - Tacrolimus