The Destombes-Rosai-Dorfman Disease or Sinus Histiocytosis with Massive Lymphadenopathy: A Rare Case Described in the Paediatrics Department of Yalgado Ouedraogo University Hospital
| Autor: | Flore Ouédraogo, Fla Kouéta, Chantal Gabrielle Bouda, Caroline Yonaba, Laure Toguyeni, Angèle Kalmogho, Chantal Zoungrana-Ouattara, Rolande Kaboré |
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| Rok vydání: | 2017 |
| Předmět: |
Pediatrics
medicine.medical_specialty business.industry Plasmacytosis Sinus Histiocytosis with Massive Lymphadenopathy medicine.disease 030218 nuclear medicine & medical imaging Emperipolesis 03 medical and health sciences Histiocytosis 0302 clinical medicine 030220 oncology & carcinogenesis Etiology Medicine business Pathological Histiocyte Rosai–Dorfman disease |
| Zdroj: | Open Journal of Pediatrics. :345-352 |
| ISSN: | 2160-8776 2160-8741 |
| DOI: | 10.4236/ojped.2017.74039 |
| Popis: | Destombes-Rosai-Dorfman’ disease (DRD) is an inflammatory non-langerhansian histiocytosis with adenomegaly. It is a rare histiocytic disorder worldwide, less than 1000 reported cases. It is of unknown etiology and is characterized in its classical form by multiple adenomegalia, especially localized at cervical area, but also at mediastinal, axillary and inguinal areas. It is a benign condition, even if it is deforming, with spontaneously resolving evolution. We report a case of DRD disease in a 13-year-old girl, hospitalized in the Department of Pediatrics of the Yalgado Ouedraogo University Hospital Center in Ouagadougou, in March 2015. She was admitted for a voluminous bilateral painless cervical swelling, with no sign of local compression, having been operating for about a year. Biological tests showed signs of chronic inflammation; the imagery specified the benign character of these formations: adenomegalia. Confirmation of the diagnosis was made by pathological examination, describing the aspect of emperipolesis to histology and, immunohistochemistry, polytypic plasmacytosis and PS100 positive/CD1a histiocytosis negative. The treatment could not be properly carried out, in view of the early discharge, against medical advice from the patient. The evolution would have been marked, according to the parents, by a slight involution of swelling. |
| Databáze: | OpenAIRE |
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