Published online Jul 26, 2021. doi: 10.12998/wjcc.v9.i21.6032
Peer-review started: March 15, 2021
First decision: April 4, 2021
Revised: April 14, 2021
Accepted: May 24, 2021
Article in press: May 24, 2021
Published online: July 26, 2021
Rosai–Dorfman disease (RDD) is a rare histiocytic proliferation of unknown etiology commonly found in children and adolescents. The common manifestation of RDD is massive and painless bilateral cervical lymphadenopathy with extranodal disease. While extranodal involvement in RDD is common, the spleen is an infrequent site of disease.
We report a 10-mo-old female infant with RDD presenting multiple splenic masses without cervical lymphadenopathy. She had fever, and blood tests showed leukocytosis, anemia, and elevated erythrocyte sedimentation rate and C-reactive protein. Ultrasound, computed tomography, and magnetic resonance images demonstrated multiple splenic masses. Despite antibiotic therapy, her symptoms were not relived. She underwent diagnostic splenectomy and was discharged with recovery.
In pediatric patients with refractory infectious symptoms or hematological abnormalities, clinicians should suspect RDD, even in patients without significant lymphadenopathy.
Core Tip: Rosai–Dorfman disease (RDD) is a rare histiocytic proliferation of unknown etiology, characterized by massive and painless bilateral cervical lymphadenopathy accompanied by fever, commonly found in children and adolescents. The spleen is an infrequent site of extranodal involvement in RDD. We report a 10-mo-old girl with RDD with extranodal involvement of the spleen without significant cervical lympha