J Rheum Dis.  2014 Aug;21(4):196-200. 10.4078/jrd.2014.21.4.196.

Delayed and Long-term Remission of Refractory Hemolytic Anemia in a Child with Systemic Lupus Erythematosus Treated with Rituximab

Affiliations
  • 1Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea. jsoh@uuh.ulsan.kr
  • 2Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.

Abstract

Autoimmune hemolytic anemia (AIHA) is a relatively common cause of anemia in children and adults with systemic lupus erythematosus (SLE). Although AIHA responds to steroids, in case of refractory or steroid-dependent AIHA, immunosuppressants and intravenous immunoglobulin have been used as second line agents. Rituximab, an anti-CD20 monoclonal antibody, is emerging in the treatment of SLE refractory to conventional therapy. Herein, we report a case of delayed and sustained remission of refractory hemolytic anemia in a child with SLE, post rituximab treatment. A 12-year-old female child with dizziness was referred to our department and was diagnosed with SLE combined with hemolytic anemia and renal tubular acidosis. Since frequent relapse of hemolytic anemia had occurred during the steroid tapering course, even though she had been treated with additional immunosuppressants (azathioprine, mycophenolate mofetil), the patient received 2 doses of rituximab 500 mg at 2 weeks interval at 18 months post diagnosis. After 15 months of rituximab administration, her anemia and renal tubular acidosis were fully recovered, enough to stop all medications. She remained well without recurrence for up to 3 years and 4 months after rituximab treatment.

Keyword

Systemic lupus erythematosus; Hemolytic anemia; Rituximab; Steroid

MeSH Terms

Acidosis, Renal Tubular
Adult
Anemia
Anemia, Hemolytic*
Anemia, Hemolytic, Autoimmune
Child*
Diagnosis
Dizziness
Female
Humans
Immunoglobulins
Immunosuppressive Agents
Lupus Erythematosus, Systemic*
Recurrence
Steroids
Rituximab
Immunoglobulins
Immunosuppressive Agents
Steroids

Figure

  • Figure 1. Kidney ultrasonography reveals diffuse increased echogenecity of medulla of both kidneys, suggesting medullary nephrocalcinosis.

  • Figure 2. Trends of hemoglobin and total bilirubin levels before and after rituximab treatment. The bar graph stands for daily amounts of oral steroid calculated in terms of prednisolone. Hemoglobin level increased, and total bilirubin level and the doses of oral steroid decreased after the use of rituximab. SLE, systemic lupus erythematosus; HA, hemolytic anemia.


Reference

1. Lee SG, Yoo B, Kim KM, Choi HO, Oh JS, Nah SS, et al. Successful Treatment of Neuropsychiatric Syndrome with Rituximab in a Patient with Systemic Lupus Erythematosus and Dermatomyositis Overlap Syndrome. J Korean Rheum Assoc. 2008; 15:170–4.
Article
2. Wang SW, Cheng TT. Systemic lupus erythematosus with refractory hemolytic anemia effectively treated with cyclosporin A: a case report. Lupus. 2005; 14:483–5.
3. Tokunaga M, Saito K, Nakatsuka K, Nakayamada S, Nakano K, Tsujimura S, et al. Successful treatment of intravenous cyclophosphamide pulse therapy for systemic lupus erythematosus complicated with steroid-resistant hemolytic anemia. Nihon Rinsho Meneki Gakkai Kaishi. 2003; 26:304–9.
Article
4. Sarles HE, Levin WC. The role of splenectomy in the management of acquired autoimmune hemolytic anemia complicating systemic lupus erythematosus. Am J Med. 1959; 26:547–54.
Article
5. Hwang KW, Ahn YS, Moon JY, Kim IY, Park YE, Kim GT, et al. Experience of Rituximab Treatment in Two Patients with Severe Systemic Lupus Erythematosus. J Korean Rheum Assoc. 2006; 13:230–5.
6. Reff ME, Carner K, Chambers KS, Chinn PC, Leonard JE, Raab R, et al. Depletion of B cells in vivo by a chimeric mouse human monoclonal antibody to CD20. Blood. 1994; 83:435–45.
Article
7. Leandro MJ, Cambridge G, Edwards JC, Ehrenstein MR, Isenberg DA. B-cell depletion in the treatment of patients with systemic lupus erythematosus: a longitudinal analysis of 24 patients. Rheumatology (Oxford). 2005; 44:1542–5.
Article
8. Looney RJ, Anolik JH, Campbell D, Felgar RE, Young F, Arend LJ, et al. B cell depletion as a novel treatment for systemic lupus erythematosus: a phase I/II dose-esca-lation trial of rituximab. Arthritis Rheum. 2004; 50:2580–9.
Article
9. Leandro MJ, Edwards JC, Cambridge G, Ehrenstein MR, Isenberg DA. An open study of B lymphocyte depletion in systemic lupus erythematosus. Arthritis Rheum. 2002; 46:2673–7.
Article
10. Bussone G, Ribeiro E, Dechartres A, Viallard JF, Bonnotte B, Fain O, et al. Efficacy and safety of rituximab in adults' warm antibody autoimmune haemolytic anemia: retrospective analysis of 27 cases. Am J Hematol. 2009; 84:153–7.
Article
11. Dierickx D, Verhoef G, Van Hoof A, Mineur P, Roest A, Triffet A, et al. Rituximab in autoimmune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study. J Intern Med. 2009; 266:484–91.
Article
12. Kumar S, Benseler SM, Kirby-Allen M, Silverman ED. B-cell depletion for autoimmune thrombocytopenia and autoimmune hemolytic anemia in pediatric systemic lupus erythematosus. Pediatrics. 2009; 123:e159–63.
Article
13. Oh HJ, Yun MJ, Lee ST, Lee SJ, Oh SY, Sohn I. Evans syndrome following longstanding Hashimoto's thyroiditis and successful treatment with rituximab. Korean J Hematol. 2011; 46:279–82.
Article
14. Park CY, Chung CH. A patient with mixed type Evans syndrome: efficacy of rituximab treatment. J Korean Med Sci. 2006; 21:1115–6.
Article
15. Lee JH, Lee KS. A Case of Autoimmune Hemolytic Anemia Treated with Rituximab in a Child. Korean J Hematol. 2006; 41:321–5.
Article
16. Smith KG, Jones RB, Burns SM, Jayne DR. Long-term comparison of rituximab treatment for refractory systemic lupus erythematosus and vasculitis: Remission, relapse, and retreatment. Arthritis Rheum. 2006; 54:2970–82.
Article
17. Ng KP, Cambridge G, Leandro MJ, Edwards JC, Ehrenstein M, Isenberg DA. B cell depletion therapy in systemic lupus erythematosus: long-term follow-up and predictors of response. Ann Rheum Dis. 2007; 66:1259–62.
Article
Full Text Links
  • JRD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr