Background: 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.
Case summary: 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.
Conclusion: In pediatric patients with refractory infectious symptoms or hematological abnormalities, clinicians should suspect RDD, even in patients without significant lymphadenopathy.
Keywords: Case report; Computed tomography; Magnetic resonance imaging; Rosai–Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Ultrasonography.
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