Lymphoproliferative disorders in paediatric rheumatic diseases. A report of two cases.

Ly m p h o p roliferative disorders (LPD) are reported with a much lower fre quency in children with rheumatic diseases than in their adult counterparts. We describe 2 patients who developed a lymphoma during the course of the disease. The first is a 16-year-old girl diagnosed with systemic juvenile idiopathic arthritis 6 years before who developed a mucosa-associated lym phoid tissue (MALT) lymphoma. The second report involves a boy diagnosed with systemic lupus erythematosus at 9 years of age who developed a Hodg kin’s lymphoma 9 years after the disease onset. In spite of the low frequency of LPD in children with rheumatic diseases, these processes do occur. Case 1 A 10-year-old Caucasian female presented in March 1990 with high spiking fevers, evanescent rash, and arthri tis of her right knee. Laboratory inves tigations revealed ESR of 75 mm/h and negative RF and ANA. A diagnosis of systemic onset juvenile idiopathic arth ritis (JIA) was made. The disease fol lowed a polyarticular course which re quired treatment with prednisone during 46 months (cumulative dose 8,670 mg) and methotrexate (MTX) for 6 years (cumulative dose 3,070 mg). No other potential immunosuppressive drugs were used. In May 1996, while on MTX she developed a painless, firm mass over her right parotid gland. A fine-needle aspi ration biopsy revealed reactive lymphadenitis. By March 1997 the mass had increased. A CT showed multiple e n l a r ged nodes in her right parotid area, which led to surgical excision of the parotid. Histopathology revealed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma. MTX was discontinued at that time. Serology was consistent with a past Epstein-Barr virus (EBV) infection. She had not shown xerophthalmia nor xerostomia, and her anti-Ro/SS-Aand anti-La/SS-B antibodies were negative. She received local radiotherapy and her arthritis has been adequately controlled with NSAID and intra-articular steroids.