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肥大细胞增生症(mastocytosis,MT)是由于异常的肥大细胞克隆性增殖并积聚于一个或多个系统器官(如皮肤、骨髓、肝、脾、淋巴结和胃肠道等)而引发的一组高度异质性的少见的肿瘤性疾病[1-7]。2008年WHO将其分为皮肤型MT(cutaneous mastocytosis,CM)和系统型MT(systemic mastocytosis,SM)两大类[1,6,8]。CM多见于儿童,SM多见于成人[2-5]。由于MT诊断有赖于病灶组织免疫病理学检查,而与成人相比,儿童入侵性检查执行的比较少,因此儿童MT的主要临床表现、实验室检查、累及脏器、病变严重性及预后等研究均落后于成人MT[9]。现对
Mastocytosis (MT) is a set of heights induced by the abnormal proliferation of mast cells that accumulate in one or more of the systemic organs (such as the skin, bone marrow, liver, spleen, lymph nodes, gastrointestinal tract, etc.) Heterogeneous rare neoplastic disease [1-7]. In 2008, WHO divided it into two categories: cutaneous mastocytosis (CM) and systemic mastocytosis (SM) [1,6,8]. CM more common in children, SM more common in adults [2-5]. Because MT diagnosis depends on the immunopathological examination of the lesion tissue, and compared with adults, the implementation of children’s invasive examination is relatively small, so the main clinical manifestations of MT, laboratory tests, organ involvement, severity of disease and prognosis All lagging behind adult MT [9]. Right now