29岁,脂质肉芽肿病Erdheim-Chester【脂质肉芽肿病】

发布网友 发布时间：2024-10-24 16:49

我来回答

共1个回答

热心网友 时间：2024-10-29 13:59

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis first described by Jakob Erdheim and William Chester in 1930. They described two patients who had a distinctive lipidosis with associated bone changes. In 1972, Jaffe reported a third patient and coined the term "Erdheim-Chester disease". Jaffe described the disease as a rare histiocytic disorder of alts characterized by an infiltrate of lipid-laden macrophages, multinucleated giant cells, inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow and a generalized sclerosis of the long bones sparing the epiphysis. Approximately 80 cases have been reported in the literature. Erdheim-Chester Disease versus Langerhans Cell HistiocytosisECD is very similar to Langerhans cell histiocytosis (LCH) in its histopathology and distribution and was thought to represent the same disease process. ECD differs from LCH in its age distribution, immunochemical characteristics and radiologic characteristics. LCH is a disease of childhood. The average age at presentation for ECD is 53 years. The Langerhans cell histiocytes stain positively for S-100 antigen and have Birbeck granules on electron microscopy. The histiocytes in ECD do not share these characteristics. The bony lesions in ECD are typically symmetric and sclerotic and involve the long bones in the region of the metadiaphyses. The lesions of LCH are typically lytic and involve the axial skeleton.

（湘雅医院施为大夫郑重提醒：因不能面诊患者，无法全面了解病情，以上建议仅供参考，具体诊疗请一定到医院在医生指导下进行！）

参考资料：湘雅医院施为 http://shiwei1.haodf.com/