Open AccessPeriodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, Hodgkin's lymphoma,and the familial Mediterranean fever gene
Author(s) -
Ala Atamna,
Corina Hershkovici,
Avishay Elis
Publication year - 2014
Publication title -
case reports in internal medicine
Language(s) - English
Resource type - Journals
eISSN - 2332-7251
pISSN - 2332-7243
DOI - 10.5430/crim.v1n1p37
Subject(s) - familial mediterranean fever , pharyngitis , mefv , medicine , adenitis , stomatitis , lymphoma , recurrent aphthous stomatitis , gene mutation , dermatology , immunology , mutation , gene , disease , genetics , biology
The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) is a benign, self-limited, auto-inflammatory disorder. The attacks usually start at age 2 to 5 years and cease before the age of 10 years. Affected children grow and develop normally and experience no long-term sequelae. The course of PFAPA syndrome is attenuated by the carrier state of the familial Mediterranean fever (FMF) gene, MEFV. A high rate of MEFV mutations has also been reported in various hemato-lymphoid neoplasms. We describe a 19-year-old woman with PFAPA syndrome in remission who was diagnosed with Hodgkin's lymphoma. Although mutations in the FMF gene were not found in this case, the association of PFAPA syndrome with FMF gene mutations and hematological malignancies is discussed.