[Successful treatment of intravenous cyclophosphamide pulse therapy for systemic lupus erythematosus complicated with steroid-resistant hemolytic anemia]

Nihon Rinsho Meneki Gakkai Kaishi. 2003 Oct;26(5):304-9. doi: 10.2177/jsci.26.304.
[Article in Japanese]

Abstract

(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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adolescent
  • Anemia, Hemolytic, Autoimmune / drug therapy*
  • Anemia, Hemolytic, Autoimmune / etiology
  • Cyclophosphamide / administration & dosage*
  • Female
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Injections, Intravenous
  • Lupus Erythematosus, Systemic / complications*
  • Middle Aged
  • Pulse Therapy, Drug

Substances

  • Immunosuppressive Agents
  • Cyclophosphamide