| Popis: |
Background and aim Marginal zone lymphomas represent 3% of all lymphomas and are divided into extranodal (ENMZL), splenic and nodal. ENMZL is characterised by indolent natural history, is usually diagnosed in localised stages (70%) and chronic antigen stimulation seems to be involved in its pathogenesis. Typically seen in the 6th decade of life, it is responsible for 8% of adult non-Hodgkin lymphoma. According to multicentre NHL-BFM studies, the incidence of ENMZL in children is 0.1%, most commonly found in the gastrointestinal tract (44%). The salivary gland is involved only in 5% of paediatric patients, being preceded in 1/3 cases by Sjogren syndrome (SS). Herein, we report a 15-year-old girl with advanced ENZML of salivary glands following SS, a very rare diagnosis in children. METHOD AND Results Female patient, 13y, presented with enlarged jaw area, was evaluated by an oral-maxillofacial surgeon and cherubism was excluded. Laterocervical ultrasound, head and neck MRI: hypertrophic parotid glands with cystic images, small adenopathies. Based on parotid swelling, dry mouth and eyes, bilateral hypo-lacrimation on Schirmer’s test, positive anti-Ro, anti-La and anti-nuclear antibodies, she was diagnosed with primary SS. At age 15: progressive parotid swelling, no ‘B’ symptoms, leucopenia 3.38 × 10^9/l, anaemia 10.5 g/dl, sedimentation rate 33 mm/h, LDH 225UI (n Conclution ENMZL of parotid glands following SS is rarely seen, especially in paediatric patients, thus the knowledge about this lymphoma type is limited. It is low-grade and responds well to R-CHOP chemotherapy, even in advanced stages. Nevertheless, regular follow-up is required, as relapses can occur during the course of the disease. |
