勒-雪(Letterer-Siwe)氏病是一种发生于婴幼儿的非脂质性组织细胞增生症。也有人称之为急性分化性组织细胞增生症,或把本病与罕-叔-克(Hand-Schuller-Cbristian)氏症、骨嗜酸性肉芽肿三者合在一起,总称之为组织细胞增生病X(histio-eytosis X )。本病比较少见,国内文献自1957年开始有少数病例报道。现将近来我们遇到的一例简要报告如下: 病例报告患儿为18个月的女孩。二十天前因饮食不适,加之受凉,曾呕吐数次,继而发热,体温波动在39℃左右。曾到合作医疗站及卫生所求诊,按上感、消化不良作对症处理。1981年4月8日,因症状加重,入县医院治
Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarte...
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Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki- 67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH.
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