TY - JOUR T1 - Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome JO - Reumatología Clínica (English Edition) T2 - AU - Collado,Paz AU - Kelada,Aml AU - Cámara,Maria AU - Zeft,Andrew AU - Flagg,Aron SN - 21735743 M3 - 10.1016/j.reumae.2017.01.011 DO - 10.1016/j.reumae.2017.01.011 UR - https://www.reumatologiaclinica.org/en-extranodal-marginal-zone-b-cell-articulo-S2173574318300595 AB - Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined.We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy.To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS. ER -