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Case Report
A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine)
Clin Pediatr Hematol Oncol 2019;26:99-104.
Published online October 31, 2019
© 2019 Korean Society of Pediatric Hematology-Oncology

Min Hyung Cho1, Hyun Joo Jung1, Jae Ho Han2 and Jun Eun Park1

Departments of 1Pediatrics and 2Pathology, Ajou University School of Medicine, Suwon, Korea
Correspondence to: Jun Eun Park
Department of Pediatrics, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon 16499, Korea
Tel: +82-31-219-5168
Fax: +82-31-219-5169
Received September 16, 2019; Revised October 4, 2019; Accepted October 7, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.
Keywords: Rosai-Dorfman disease, 2-Chlorodeoxyadenosine, Refractory, Recurrent
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October 2019, 26 (2)
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  • Jun Eun Park