지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아혈액종양학회 Clinical Pediatric Hematology-Oncology Clinical Pediatric Hematology-Oncology Vol.26 No.2
- 발행연도
- 2019.1
- 수록면
- 99 - 104 (6page)
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초록· 키워드
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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.
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참고문헌 (15)
참고문헌 신청
Emile JF / 2016 / Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages / Blood 127:2672~2681
Foucar E / 1990 / Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity / Semin Diagn Pathol 7:19~73
Pulsoni A / 2002 / Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review / Am J Hematol 69:67~71
Rosai J / 1969 / Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity / Arch Pathol 87:63~70
Guchelaar HJ / 1994 / Clinical and toxicological aspects of the antineoplastic drug cladribine: a review / Ann Hematol 69:223~230
Foucar E / 1990 / Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity / Semin Diagn Pathol 7:19~73
Pulsoni A / 2002 / Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review / Am J Hematol 69:67~71
Rosai J / 1969 / Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity / Arch Pathol 87:63~70
Guchelaar HJ / 1994 / Clinical and toxicological aspects of the antineoplastic drug cladribine: a review / Ann Hematol 69:223~230
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