Niramitmahapanya Sathit, Deerochanawong Chaicharn, Suwanbun Tawatchai, Serirat Onsiri, Utrapichart Usakorn, Kalayanarooj Siripan, Kuakpaetoon Thiti
Department of Medicine, Rajavithi Hospital, College of Medicine, Rangsit University, Bangkok, Thailand.
J Med Assoc Thai. 2011 Mar;94 Suppl 2:S135-9.
A 19-year-old Thai man presented with symptoms of central diabetes insipidus, exopthalmos and multiple ostolytic lesions of the cranial vault. Skin biopsy showed strongly positive CD 1a and S100. Electron microscopy showed rod shape and pentalaminar granules called "Birbeck" granules, a diagnosis compatible with Langerhans' cell histiocytosis (LCHs) or histiocytosis X. He was successfully treated with systemic chemotherapy.
一名19岁的泰国男子出现中枢性尿崩症、眼球突出及颅顶多处溶骨性病变的症状。皮肤活检显示CD 1a和S100呈强阳性。电子显微镜检查显示有杆状和五板层颗粒,称为“伯贝克”颗粒,诊断符合朗格汉斯细胞组织细胞增多症(LCHs)或组织细胞增多症X。他接受全身化疗后获得成功治疗。