(Case 1) A 13-years-old female had multiple
arthralgia and butterfly rush, when she admitted in our hospital in May 2001. Nephropathy,
hemolytic anemia (Hb 6.3 g/dl and direct Coombs 3+) and high titers of
antinuclear antibodies and anti-
ds-DNA antibody were disclosed and she was diagnosed as
systemic lupus erythematosus (SLE). Although combination
therapy of PSL 60 mg/day with a
steroid pulse
therapy,
cyclosporine or an
immunosorbent treatment, severe
hemolytic anemia remained. However, monthly
cyclophosphamide pulse
therapy (IV-CY), which was started for the
steroid-resistant
hemolytic anemia, has gradually become effective and Hb improved up to 11.4 g/dl after 6 courses of IV-CY. (Case 2) A 53-years-old woman diagnosed as SLE in 1978 and she had PSL 5 mg for over 10 years. Severe
anemia (Hb 5.9 g/dl) was disclosed with a slight
fever in June 2001, and she admitted in our hospital for further examinations. Progressive
hemolytic anemia was revealed with marked decrease of Hb (3.4 g/dl) and high titer of direct Coombs (3+). Neither PSL (50 mg/day) nor
steroid pulse
therapy were effective against
hemolytic anemia. In contrast, 3 courses of monthly IV-CY (500 mg/day) resulted in the resolution of
hemolysis. It is well known that the
steroid-resistant
hemolytic anemia is extremely hard to treat and leads to miserable prognosis, but we here propose IV-CY as an alternative and invaluable choice for the treatment of refractory
hemolytic anemia complicated with SLE.