[Hemophagocytic syndrome as primary manifestation in a patient with systemic lupus erythematosus after parturition].
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.
Yoshida
Shuzo
S
First Department of Internal Medicine, Osaka Medical College, Takatsuki, Osaka.
Takeuchi
Tohru
T
Itami
Yasuo
Y
Hata
Kenichiro
K
Watanabe
Koko
K
Shoda
Takeshi
T
Kotani
Takuya
T
Makino
Shigeki
S
Hanafusa
Toshiaki
T
D000328:Adult; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D011247:Pregnancy; D011644:Puerperal Disorders