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

• Park JH
• Im JW
• Jun HK
• Park HM
• Choi SW
• Park SK
• Oh JS

• KMID: 2222961
• DOI: http://doi.org/10.4078/jrd.2014.21.4.196

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

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

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  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.

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