Japanese Journal of Clinical Immunology
Online ISSN : 1349-7413
Print ISSN : 0911-4300
ISSN-L : 0911-4300
Case Reports
Hemophagocytic syndrome as primary manifestation in a patient with systemic lupus erythematosus after parturition.
Shuzo YOSHIDATohru TAKEUCHIYasuo ITAMIKenichiro HATAKoko WATANABETakeshi SHODATakuya KOTANIShigeki MAKINOToshiaki HANAFUSA
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2009 Volume 32 Issue 1 Pages 66-70

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Abstract

  A 33-year-old woman presented with fever, malar rash, lymphadenopathy and pancytopenia 3 weeks after parturition. Serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody and anti-SS-A antibody were positive. Serum ferritin level was elevated (3454 ng/ml), and bone marrow aspirate revealed hemophagocytosis. She was diagnosed having systemic lupus erythematosus (SLE) associated with hemophagocytic syndrome (HPS). Oral prednisolone (55 mg/day) was initiated. Clinical manifestations and pancytopenia were improved and serum ferritin level was decreased. However, elevated anti-dsDNA antibody titer and reduced C3 level continued for a month after steroid therapy. The additional therapy of tacrolimus (3 mg/day) improved clinical and laboratory findings. This is a rare SLE case associated with HPS as primary manifestation after parturition.

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© 2009 The Japan Society for Clinical Immunology
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