摘要目的:分析系统性肥大细胞增生症（SM）的临床病理特点，提高对该病的认识。方法:报道l例中年男性SM患者的临床表现及诊疗经过，并进行多学科讨论。结果:中年男性，全身骨痛，发现甲状腺结节，多处淋巴结肿大，影像学检查拟诊为甲状腺恶性肿瘤伴淋巴结及骨转移，病理结果示骨小梁间可见片状增生细胞，胞质透亮，核形态欠规则；甲苯胺蓝染色示肥大细胞（+），免疫组织化学CD117、CD2示增生肥大细胞（+）；诊断为SM，广泛累及骨髓。给予沙利度胺、骨化三醇胶丸治疗。结论:认识SM的疾病特征有助于临床正确诊断及治疗。

abstractsObjective:By analyzing the clinical and pathologic manifestations of systemic mastocytosis (SM) to improve the recognition of the disease.Methods:Clinical manifestations, diagnosis and treatment of a middle-aged male patient with SM was reported with multidisciplinary discussions.Results:A middle-aged man with bone pain, thyroid nodules and lymphadenectasis came to our clinic. Thyroid cancer with lymph node and bone metastasis was suspected by imaging examination. The pathological results showed cell proliferation with transparent cytoplasm and irregular nuclear in the trabecular bone. Toluidine blue staining showed the proliferated cells were mast cells(+). Immunohistochemistry showed proliferating mast cells stained with CD117 and CD2. SM with extensive bone marrow involvement was diagnosed and treated with thalidomide and calcitriol.Conclusion:Knowing the characteristics of SM is helpful for accurate diagnosis and treatment.