Reumatología Clínica (English Edition) Reumatología Clínica (English Edition)
Case Report
Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome
Linfoma no Hodgkin de linfocitos B de la zona marginal, extranodal: una complicación inesperada en niños con síndrome de Sjögren
Paz Colladoa,,, , Aml Keladab,, Maria Cámarac, Andrew Zeftd, Aron Flaggb
a Department of Rheumatology/Paediatric Rheumatology Unit, Hospital Universitario Severo Ochoa, Madrid, Spain
b Department of Pediatric Hematology/Oncology and Blood & Marrow Transplant, Cleveland Clinic, OH, USA
c Pathologic Anatomy Department, Hospital Universitario Severo Ochoa, Madrid, Spain
d Center for Pediatric Rheumatology and Immunology Cleveland Clinic, OH, USA
Received 09 November 2016, Accepted 26 January 2017

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.


El síndrome de Sjögren (SS) es una enfermedad sistémica autoinmune caracterizada por la infiltración de linfocitos en glándulas exocrinas, provocando el típico síndrome seco. A diferencia de los adultos, el SS es una afección rara en niños, y el riesgo de malignización no ha sido descrito.

Describimos la detección de linfoma B de la zona marginal extranodal de tejido linfoide asociado a mucosa (linfoma MALT) en 2 niños con SS. La aspiración con aguja fina de las glándulas salivares (SG) mostró hallazgos inespecíficos que retrasaron el diagnóstico de SS. El diagnóstico de linfoma B asociado a SS juvenil se realizó con base en los hallazgos morfológicos e inmunohistoquímicos detectados en biopsia.

Hay que tomar conciencia de que un linfoma B puede acontecer en el curso de un SS como una complicación inesperada en niños, principalmente para realizar una derivación correcta a oncología.

Sjögren's syndrome, Child, B-cell lymphoma
Palabras clave
Síndrome de Sjögren, Niño, Linfoma de células B


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