Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892; email:
Tel Hai College, Upper Galilee, 1220800 Israel; email:
Annu Rev Pathol. 2017 Jan 24;12:487-514. doi: 10.1146/annurev-pathol-052016-100312.
Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in the molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations, including new and novel tyrosine kinase inhibitors.
系统性肥大细胞增多症是一种肥大细胞的克隆性疾病，其特征性皮损、肥大细胞介质释放发作和造血功能紊乱的表现可有差异。目前尚无治愈性治疗方法。传统的治疗方法依赖于拮抗肥大细胞释放的介质、抑制介质分泌或调节肥大细胞增殖的药物。对系统性肥大细胞增多症病理生理学的分子认识的最新进展提供了新的治疗考虑因素，包括新型酪氨酸激酶抑制剂。
