p53 expression in biopsies from children with Langerhans cell histiocytosis
p53 expression in biopsies from children with Langerhans cell histiocytosis
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DOI：
10.1097/00043426-200212000-00010
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发表时间：
2002-12-01
影响因子：
1.2
通讯作者：
Petersen, BL
中科院分区：
文献类型：
--
作者：
Bank, MI;Rengtved, P;Petersen, BL
目的：朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的儿童和成人疾病，可引起皮疹、溶骨性骨病变、各器官肿瘤生长，部分患者可引起器官功能障碍。该疾病的病因尚不清楚，也不清楚为什么有些患者只发生单系统病变而不复发，而另一些患者则发生致命的多器官疾病。免疫组织化学检测p53肿瘤抑制基因产物的表达可作为DNA修复控制和细胞凋亡变化的指导。作者选择在LCH患儿的活检中分析p53的表达，并将其与广泛器官的临床表现和结果联系起来。患者和方法：研究对象为32例儿童（男孩19例，女孩13例），50例标本，中位年龄31又1/4岁，5个月至12又1/3岁，CD1a阳性确诊为LCH。病理朗格汉斯细胞（pLC） p53阳性率分别为0% ~ 20%、20% ~ 40%、40% ~ 60%、60% ~ 80%和80% ~ 100%，用1 ~ 5的分级系统对载玻片进行p53抗体染色和半定量评估。结果：p53蛋白在所有病变的pLC中均有不同程度的表达。p53的表达程度与临床表现和预后均无相关性。结论：在pLC中p53表达的增加表明DNA修复控制的改变，伴有或不伴有细胞凋亡的异常控制。
Purpose: Langerhans cell histiocytosis (LCH) is a rare pediatric and adult disease causing skin rashes, osteolytic bone lesions, tumorous growth in various organs, and in some patients, organ dysfunction. The cause of the disease is obscure, and it is not yet understood why some patients develop single-system lesions only without relapse, whereas others develop fatal multiorgan disease. The expression of p53 tumor suppressor gene product detected immunohistochemically can be used as a guideline to alterations in DNA repair control and apoptosis. The authors have chosen to analyze p53 expression in biopsies from children with LCH and correlate it with clinical manifestation and outcome in a broad range of organs.Patients and Methods: The study was performed on 50 specimens from 32 children (19 boys and 13 girls), median age 3 1/4 years, range 5 months to 12 1/3 years with a definite diagnosis of LCH based on CD1a positivity. The slides were stained with p53 antibody and semiquantitatively evaluated using a grading system from 1 to 5 as an estimate for 0% to 20%, 20% to 40%, 40% to 60%, 60% to 80%, and 80% to 100% p53-positive for pathologic Langerhans cells (pLC), respectively.Results: The p53 protein was expressed in various degrees in pLC in all lesions. The degree of p53 expression could not be correlated to either clinical manifestation or outcome. Conclusions: An increased expression of p53 in pLC indicates an altered DNA repair control with or without abnormal control of apoptosis.