Rituximab Treatment in a Child with Rosai-Dorfman Disease and Systemic Lupus Erythematosus
2010
To the Editor:
Rosai-Dorfman disease (RDD) is a rare disorder in childhood. The association of RDD and systemic lupus erythematosus (SLE) is very rare, with only 2 previously reported cases, and neither of these in a child1,2. We describe a child who presented with histopathology-confirmed RDD, and developed SLE 3 years later.
A 7-year-old boy of Canadian First Nations background presented at age 4 years to the pediatric hematology/oncology clinic with a 3-month history of asymptomatic, bilateral massive lymphadenopathy involving the cervical, supraclavicular, mediastinal, hilar, and axillary lymph nodes. He has a healthy fraternal twin, and there is no family history of connective tissue diseases. The lymphadenopathy failed to respond to a course of antibiotics. Laboratory tests showed mild microcytic hypochromic anemia with lymphopenia [white blood cells (WBC) 6.29 x 109/l, hemoglobin 103 g/l, mean corpuscular volume 74.3 fl, mean corpuscular hemoglobin 23.5 pg/cell, platelets 262 x 109/l, lymphocytes 0.63 x 109/l, absolute neutrophil count 4.98 x 109/l) and high erythrocyte sedimentation rate, 63 mm/h. Serial chest radiographs showed persistent nonprogressive large bilateral mediastinal, hilar, right infrahilar, and paratracheal lymphadenopathy (Figure 1). Computed tomography scan showed adenopathy, airway narrowing, and atelectasis as well as superior vena cava displacement (Figure …
Address correspondence to Dr. Alqanatish; E-mail: jqanatish{at}hotmail.com
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