title: Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.
abstract: 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.
authors.affiliation: Department of Rheumatology/Paediatric Rheumatology Unit, Hospital Universitario Severo Ochoa, Madrid, Spain. Electronic address: paxko10@gmail.com.
authors.forename: Paz
authors.initials: P
authors.lastname: Collado
authors.affiliation: Department of Pediatric Hematology/Oncology and Blood & Marrow Transplant, Cleveland Clinic, OH, USA.
authors.forename: Aml
authors.initials: A
authors.lastname: Kelada
authors.affiliation: Pathologic Anatomy Department, Hospital Universitario Severo Ochoa, Madrid, Spain.
authors.forename: Maria
authors.initials: M
authors.lastname: Cámara
authors.affiliation: Center for Pediatric Rheumatology and Immunology Cleveland Clinic, OH, USA.
authors.forename: Andrew
authors.initials: A
authors.lastname: Zeft
authors.affiliation: Department of Pediatric Hematology/Oncology and Blood & Marrow Transplant, Cleveland Clinic, OH, USA.
authors.forename: Aron
authors.initials: A
authors.lastname: Flagg
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications / Q000175:diagnosis*; D008297:Male; D012859:Sjogren's Syndrome / Q000150:complications*
keywords: B-cell lymphoma; Child; Linfoma de células B; Niño; Sjögren's syndrome; Síndrome de Sjögren