A 44-year-old female. Developed
polyarthralgia in August 2002. The patient was diagnosed with
systemic lupus erythematosus (SLE) in November, due to
polyarthralgia,
leukopenia, anti
ds-DNA antibody positive,
antinuclear antibody positive, and false positive serologic test for
syphilis. Hypocomplementemia continued even after the
steroid treatment was conducted with insufficient control. In November 2003, ran
fever and observed
polyarthralgia. In December, gross
hematuria and
purpura appeared. The patient was hospitalized on December 25.
Thrombotic thrombocytopenic purpura (
TTP) was suspected from the emergence of fragmented red cells,
hemolysis,
thrombocytopenic purpura,
headache, renal dysfunction and
fever. As
hemophagocytic syndrome (HPS) was suspected from hyper-ferritinemia, bone marrow aspiration was conducted. Macrophage confirmed hemophagocytic image and the patient was diagnosed with HPS complication.
Methylprednisolone pulse
therapy was conducted for three days, followed by the administration of
prednisolone 60 mg.
Plasma exchange therapy was also conducted from the first day of hospitalization. Recurrence of
TTP after
plasma exchange therapy, but it improved by additional
plasma exchange. Low vWF-CP (ADAMTS-13) activity was observed in this case, and anti-vWF-CP antibody was positive.
TTP and HPS are both critical intractable complications of SLE. Bearing in mind the possibility of simultaneous complication of both symptoms, prompt diagnosis is crucial for life-saving.