JRD Journal of Rheumatic Diseases J Rheum Dis 2093-940X 2233-4718 The Korean College of Rheumatology 10.4078/jrd.2014.21.4.196 jrd-21-196 Case Report Delayed and Long-term Remission of Refractory Hemolytic Anemia in a Child with Systemic Lupus Erythematosus Treated with Rituximab ParkJu Hwan 1 ImJae Wook 1 JunHong Kil 1 ParkHae Min 1 ChoiSeung Won 1 ParkSang Kyu 2 OhJi Seon 1 Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea Corresponding to: Ji Seon Oh, Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, 877 Bangeojinsunhwan-doro, Dong-gu, Ulsan 682-060, Korea. E-mail: jsoh@uuh.ulsan.kr 10 2014 31 10 2014 21 4 196 200 31052013 18072013 05082013 Copyright © 2014 The Korean College of Rheumatology 2014 This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited. REFERENCES Lee SG Yoo B Kim KM Choi HO Oh JS Nah SS Successful Treatment of Neuropsychiatric Syndrome with Rituximab in a Patient with Systemic Lupus Erythematosus and Dermatomyositis Overlap Syndrome J Korean Rheum Assoc 2008 15 1704 Wang SW Cheng TT Systemic lupus erythematosus with refractory hemolytic anemia effectively treated with cyclosporin A: a case report Lupus 2005 14 4835 Tokunaga M Saito K Nakatsuka K Nakayamada S Nakano K Tsujimura S Successful treatment of intravenous cyclophosphamide pulse therapy for systemic lupus erythematosus complicated with steroid-resistant hemolytic anemia Nihon Rinsho Meneki Gakkai Kaishi 2003 26 3049 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 54754 Hwang KW Ahn YS Moon JY Kim IY Park YE Kim GT Experience of Rituximab Treatment in Two Patients with Severe Systemic Lupus Erythematosus J Korean Rheum Assoc 2006 13 2305 Reff ME Carner K Chambers KS Chinn PC Leonard JE Raab R Depletion of B cells in vivo by a chimeric mouse human monoclonal antibody to CD20 Blood 1994 83 43545 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 15425 Looney RJ Anolik JH Campbell D Felgar RE Young F Arend LJ 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 25809 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 26737 Bussone G Ribeiro E Dechartres A Viallard JF Bonnotte B Fain O Efficacy and safety of rituximab in adults' warm antibody autoimmune haemolytic anemia: retrospective analysis of 27 cases Am J Hematol 2009 84 1537 Dierickx D Verhoef G Van Hoof A Mineur P Roest A Triffet A Rituximab in autoimmune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study J Intern Med 2009 266 48491 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 e15963 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 27982 Park CY Chung CH A patient with mixed type Evans syndrome: efficacy of rituximab treatment J Korean Med Sci 2006 21 11156 Lee JH Lee KS A Case of Autoimmune Hemolytic Anemia Treated with Rituximab in a Child Korean J Hematol 2006 41 3215 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 297082 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 125962 Figures and Tables

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

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.

Laboratory findings before and after rituximab treatment in a child with systemic lupus erythematosus and hemolytic anemia

Rituximab administration
  2008/07 2008/12 2009/04 2009/09 2009/12 2010/01 2010/02 2010/04 2010/07 2011/02 2011/09 2012/04 2012/07 2012/09 2012/12 2013/03
Hb (/dL) 7.1 10.1 5.6 8.3 6.8 12.9 10.6 8.9 9.9 11.7 13.1 13.1 13.4 14.8 13.7 13.8
CRC (%) 5.38 − 7.37 12.75 5.53 2.96 7.14 5.9 5.92 3.17 1.12 1.43 1.16 1.44 0.93 1.01
CD19 (/uL) − − − − − 165 0.4 0.3 0.7 11.4 26.8 − 312 − − −
IgA (mg/dL) − − − − − 101 − − 46 3 2 − 0 0 2 0
IgM (mg/dL) − − − − − 130 − − ＜25 ＜25 ＜25 − ＜25 ＜25 ＜25 ＜25
IgG (mg/dL) − − − − − 754 − − 579 585 557 − 376 369 355 ＜300
K (mM/L) 2.3 5.1 3.9 4.5 4.8 4.3 3.8 4.2 − 4 − 4.4 4 4 4.2 4.3
tCO2 (mmol/L) 14 23 24 23 28 28 28 25 − 29 − 23 24 26 26 25
Supplementation of potassium citrate

Hb: hemoglobin, CRC: corrected reticulocyte count, CD: Cluster of Differentiation, IgA: immunoglobulin A, IgM: immunoglobulin M, IgG: immunoglobulin G, K: potassium, tCO2: total carbon dioxide.