Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 26, 2021; 9(21): 6032-6040
Published online Jul 26, 2021. doi: 10.12998/wjcc.v9.i21.6032
Rosai-Dorfman disease in the spleen of a pediatric patient: A case report
Hwaseong Ryu, Jae-Yeon Hwang, Yong-Woo Kim, Tae-Un Kim, Joo-Yeon Jang, Su-Eun Park, Eu-Jeen Yang, Dong-Hoon Shin
Hwaseong Ryu, Jae-Yeon Hwang, Yong-Woo Kim, Tae-Un Kim, Joo-Yeon Jang, Department of Radiology, Pusan National University Yangsan Hospital, Yangsan 50612, South Korea
Su-Eun Park, Eu-Jeen Yang, Department of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, South Korea
Dong-Hoon Shin, Department of Pathology, Pusan National University Yangsan Hospital, Yangsan 50612, South Korea
Author contributions: Ryu H and Hwang JY reviewed the literature and contributed to manuscript drafting; Kim YW, Kim TU, and Jang JY reviewed the images and contributed to manuscript drafting; Park SE made the diagnosis; Yang EJ, the patient’s doctor, performed the treatment; Shin DH performed the pathological examination; Hwang JY was responsible for revising the manuscript for important intellectual content; all authors issued the final approval for the version to be submitted.
Informed consent statement: Written informed consent was obtained from the patient’s parents for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jae-Yeon Hwang, MD, PhD, Associate Professor, Department of Radiology, Pusan National University Yangsan Hospital, Geumo-ro 20, Yangsan 50612, South Korea. jyhwang79@gmail.com
Received: March 15, 2021
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
Abstract
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: Rosai–Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, Ultrasonography, Computed tomography, Magnetic resonance imaging, Case report

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 lymphadenopathy.