Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease
Rosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the...
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| Format: | Article |
| Language: | English |
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Wiley
2020-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2020/5727569 |
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| author | Volkan Karakuş Yelda Morgül Dere Dilek Ersil Soysal |
| author_facet | Volkan Karakuş Yelda Morgül Dere Dilek Ersil Soysal |
| author_sort | Volkan Karakuş |
| collection | DOAJ |
| description | Rosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment. |
| format | Article |
| id | doaj-art-a33def5a0939463a96f194cb388f7eb8 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-a33def5a0939463a96f194cb388f7eb82025-02-03T01:25:17ZengWileyCase Reports in Medicine1687-96271687-96352020-01-01202010.1155/2020/57275695727569Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman DiseaseVolkan Karakuş0Yelda Morgül Dere1Dilek Ersil Soysal2Mugla Sıtkı Kocman University Training and Research Hospital, Department of Hematology, Mentese, Mugla 48100, TurkeyMugla Sıtkı Kocman University Training and Research Hospital, Department of Pathology, Mentese, Mugla 48100, Turkeyİzmir University of Economics, Faculty of Medicine, Balçova, Izmir 35330, TurkeyRosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.http://dx.doi.org/10.1155/2020/5727569 |
| spellingShingle | Volkan Karakuş Yelda Morgül Dere Dilek Ersil Soysal Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease Case Reports in Medicine |
| title | Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease |
| title_full | Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease |
| title_fullStr | Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease |
| title_full_unstemmed | Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease |
| title_short | Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease |
| title_sort | long lasting follow up with low dose steroid in an 18 year old male with rosai dorfman disease |
| url | http://dx.doi.org/10.1155/2020/5727569 |
| work_keys_str_mv | AT volkankarakus longlastingfollowupwithlowdosesteroidinan18yearoldmalewithrosaidorfmandisease AT yeldamorguldere longlastingfollowupwithlowdosesteroidinan18yearoldmalewithrosaidorfmandisease AT dilekersilsoysal longlastingfollowupwithlowdosesteroidinan18yearoldmalewithrosaidorfmandisease |