Leguit Roos J, Wang Sa A, George Tracy I, Tzankov Alexandar, Orazi Attilio
Department of Pathology, University Medical Center Utrecht, H04-312, POB 85500, 3508 GA, Utrecht, Netherlands.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Virchows Arch. 2023 Jan;482(1):99-112. doi: 10.1007/s00428-022-03423-3. Epub 2022 Oct 10.
Mastocytosis is a neoplasm characterized by a clonal proliferation of mast cells, which accumulate in one or multiple organs, associated with an extremely heterogeneous clinical presentation. The disease can be limited to the skin (cutaneous mastocytosis) that is mostly seen in childhood and usually behaves in a benign fashion. Adult patients most often present with systemic disease with or without skin lesions. This includes indolent forms such as indolent systemic mastocytosis and its subvariant bone marrow mastocytosis, and smoldering systemic mastocytosis as well as aggressive forms including aggressive systemic mastocytosis, systemic mastocytosis with an associated myeloid neoplasm (previously called systemic mastocytosis with an associated hematologic neoplasm), and mast cell leukemia. In addition, mast cell sarcoma is a rare aggressive form of mastocytosis that can present in the skin as well as at extracutaneous sites. This review article focuses on the updates in mastocytosis of the 2022 international consensus classification (ICC).
肥大细胞增多症是一种以肥大细胞克隆性增殖为特征的肿瘤，肥大细胞积聚于一个或多个器官，临床表现极为多样。该疾病可局限于皮肤（皮肤肥大细胞增多症），多见于儿童，通常呈良性病程。成年患者最常表现为伴有或不伴有皮肤病变的系统性疾病。这包括惰性形式，如惰性系统性肥大细胞增多症及其亚型骨髓肥大细胞增多症、冒烟型系统性肥大细胞增多症，以及侵袭性形式，包括侵袭性系统性肥大细胞增多症、伴有相关髓系肿瘤的系统性肥大细胞增多症（以前称为伴有相关血液肿瘤的系统性肥大细胞增多症）和肥大细胞白血病。此外，肥大细胞肉瘤是一种罕见的侵袭性肥大细胞增多症，可出现在皮肤以及皮肤外部位。这篇综述文章重点介绍了2022年国际共识分类（ICC）中肥大细胞增多症的更新内容。
