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首页> 外文期刊>British Journal of Haematology >Malignancies and monoclonal gammopathy in Gaucher disease; a systematic review of the literature
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Malignancies and monoclonal gammopathy in Gaucher disease; a systematic review of the literature

机译：高雪氏病的恶性肿瘤和单克隆丙种球蛋白病;对文献的系统回顾

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摘要

Gaucher disease is an autosomal, recessively inherited, lysosomal storage disease, which has been associated with gammopathies and malignancies. This report represents the results of a systematic review of the literature on the prevalence of monoclonal gammopathies and malignancies in Gaucher disease. A PubMed search identified 365 studies, of which 80 reported on concomitant Gaucher disease and malignancies and/or gammopathies (15 cohort/cross sectional studies, and 65 case reports/series). Based on these studies, we conclude that compared to the general population, Gaucher patients have an increased risk of cancer in general [pooled relative risk of 1·70 (95% confidence interval 1·27-2·31)], and multiple myeloma and haematological malignancies in particular (estimated risk between 25·0 and 51·1 and 3·5 and 12·7, respectively). In addition, an increased risk has been reported for hepatocellular carcinoma and renal cell carcinoma. Several factors have been hypothesized to play a role in the pathophysiology. These include: splenectomy, immune dysregulation, endoplasmic reticulum stress, genetic modifiers, altered iron metabolism and insulin resistance.
机译：高雪氏病是一种常染色体常性，隐性遗传的溶酶体贮积病，与同种病和恶性肿瘤有关。该报告代表了有关高雪氏病中单克隆血友病和恶性肿瘤患病率文献的系统评价的结果。一项PubMed搜索确定了365项研究，其中80项报道了同时发生的Gaucher病和恶性肿瘤和/或同性恋疾病（15项队列研究/横断面研究，以及65例病例/系列报道）。根据这些研究，我们得出的结论是，与普通人群相比，Gaucher患者的总体癌症风险增加[合并相对风险为1·70（95％置信区间为1·27-2·31）]和多发性骨髓瘤尤其是血液系统恶性肿瘤（估计风险分别在25·0和51·1之间以及3·5和12·7之间）。另外，已经报道肝细胞癌和肾细胞癌的风险增加。假设有几个因素在病理生理中起作用。这些包括：脾切除术，免疫失调，内质网应激，遗传修饰因子，铁代谢改变和胰岛素抵抗。

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