Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115 USA.
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115 USA; Mastocytosis Center, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA.
Immunol Allergy Clin North Am. 2014 May;34(2):323-39. doi: 10.1016/j.iac.2014.01.010.
Mastocytosis encompasses a group of clinically and pathologically heterogeneous disorders most commonly involving the skin, which typically takes the form of urticaria pigmentosa. Mastocytosis may also involve other organs, most often bone marrow, followed by gastrointestinal tract, liver, spleen, and lymph nodes. The presence of extracutaneous involvement by mastocytosis is a major diagnostic criterion for systemic disease. However, mast cell infiltrates are often subtle in skin and extracutaneous organs, and the histologic features of mastocytosis at different anatomic sites may be variable. This article reviews the pathologic features and clinical correlates of mastocytosis involving skin and other extramedullary sites.
肥大细胞增多症包括一组临床上和病理学上异质性的疾病，最常见于皮肤，通常表现为色素性荨麻疹。肥大细胞增多症也可能涉及其他器官，最常见于骨髓，其次是胃肠道、肝脏、脾脏和淋巴结。肥大细胞增多症的皮肤外累及是全身性疾病的主要诊断标准。然而，肥大细胞浸润在皮肤和皮肤外器官中往往很细微，而且不同解剖部位的肥大细胞增多症的组织学特征可能是多变的。本文综述了累及皮肤和其他骨髓外部位的肥大细胞增多症的病理特征和临床相关性。
