肥大细胞增生症

定义

肥大细胞增多症是由于肥大细胞的克隆性、肿瘤性增殖，在一个或多个器官系统中聚集。经常发现KIT的激活性突变。它的特点是存在多灶性紧凑的集群或粘附性聚集/异常肥大细胞的浸润。这种疾病具有异质性，从可自发恢复的皮肤病变到与多器官功能衰竭和短期生存有关的高度侵袭性肿瘤。肥大细胞增多症的亚型主要通过疾病的分布和临床表现来识别。在皮肤性肥大细胞增多症（CM）中，肥大细胞浸润仍局限于皮肤，而全身性肥大细胞增多症（SM）的特点是至少有一个皮外器官受累，有或没有皮肤病变的证据。肥大细胞增多症应与肥大细胞增生症或肥大细胞激活状态严格分开，无肿瘤增生特征性形态学和/或分子学异常。

Mastocytosis is due to a clonal, neoplastic proliferation of mast cells that accumulate in one or more organ systems. Activating mutations of KIT are frequently found. It is characterised by the presence of multifocal compact clusters or cohesive aggregates/infiltrates of abnormal mast cells. The disorder is heterogeneous, ranging from skin lesions that may spontaneously regress to highly aggressive neoplasms associated with multiorgan failure and short survival. Subtypes of mastocytosis are recognised mainly by the distribution of the disease and clinical manifestations. In cutaneous mastocytosis (CM), the mast cell infiltration remains confined to the skin, whereas systemic mastocytosis (SM) is characterised by involvement of at least one extracutaneous organ with or without evidence of skin lesions. Mastocytosis should be strictly separated from mast cell hyperplasia or mast cell activation states without morphological and/or molecular abnormalities that characterize the neoplastic proliferation.

子条目 共 6 条

• 2A21.0系统性肥大细胞增生症3
• 2A21.1皮肤肥大细胞增多症3
• 2A21.2肥大细胞肉瘤
• 2A21.3皮肤外肥大细胞瘤
• 2A21.Y其他特指的肥大细胞增生症
• 2A21.Z未特指的肥大细胞增生症