Rosai-Dorfman disease and juvenile xanthogranuloma in a Thai boy: report of a case.
نویسندگان
چکیده
A 3-year-old Thai boy suffered from two histiocytoses, Rosai-Dorfman disease (RDD) and juvenile xanthogranuloma (JXG). The patient first presented with massive cervical lymphadenopathy at the age of one year. Biopsy revealed typical RDD; abnormally large CD68- and S-100 protein-positive histiocytes with occasional emperipolesis filled up the sinuses. Two years later, he developed polyuria and polydypsia. Skull film demonstrated osteolytic lesions at the occiput and left parietal region. Enlargement of the pituitary stalk was found on the magnetic resonance imaging. Despite the clinical impression of Langerhans cell histiocytosis, biopsy of the occipital lesion disclosed numerous large histiocytes with foamy cytoplasm. Several Touton giant cells with wreath-like arrangement of the nuclei were also observed. The abnormal cells expressed CD68 and factor XIIIa, but were non-reactive with S-100 protein and CD1a. Biopsy of the pituitary stalk was not performed According to the authors' literature search, this represents the first report of RDD and JXG affecting the same person.
منابع مشابه
Rosai-Dorfman Disease: A Case Report and Literature Review
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms whose etiology remains poorly elucidated. The diagnosis is based on immunohistochemistry. Its treatment is poorly defined but the prognosis is usually favorable. Here we report a 14 year old boy who presented with massive bilateral cervi...
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ورودعنوان ژورنال:
- Journal of the Medical Association of Thailand = Chotmaihet thangphaet
دوره 89 1 شماره
صفحات -
تاریخ انتشار 2006