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目的:探讨髓系肉瘤(myeloid sarcoma,MS)的临床病理学特征、免疫组织化学、鉴别诊断及预后。方法:收集16例MS患者的临床及病理资料,对其组织学形态和免疫表型、分子及预后进行回顾性研究,并分析相关文献。结果:16例MS,男9例,女7例,年龄21~72(平均45)岁,肿瘤呈弥漫片状生长,核圆形、卵圆形、染色质细腻,核分裂易见。免疫组织化学染色可表达抗髓过氧化物酶(myeloperoxidase,MPO),Lyz,CD43,CD68(kp-1)和CD117。结论:MS可能有轻微的男性优势,可能发生在任何年龄和身体的任何位置,导致多样化的临床表现及容易误诊。确诊主要依靠病理学形态及免疫组织化学。淋巴恶性肿瘤大剂量治疗的晚期并发症可以出现继发性急性髓性白血病。系统性化疗能够提高患者的总生存期。
Objective: To investigate the clinicopathological features, immunohistochemistry, differential diagnosis and prognosis of myeloid sarcoma (MS). Methods: The clinical and pathological data of 16 patients with MS were collected. The histological features, immunophenotypes, molecules and prognosis were retrospectively studied, and the related literatures were analyzed. Results: 16 MS patients were 9 males and 7 females, aged from 21 to 72 (average 45 years). The tumors grew diffusely and flaccidly. The nuclei were round and oval in shape with delicate chromatin and mitotic figures. Immunohistochemical staining can express myeloperoxidase (MPO), Lyz, CD43, CD68 (kp-1) and CD117. Conclusion: MS may have a slight male advantage and may occur anywhere in the body and in any age, leading to a wide range of clinical manifestations and misdiagnosis. The diagnosis depends mainly on pathological morphology and immunohistochemistry. Secondary complications of high-dose treatment of lymphoid malignancies may occur secondary acute myeloid leukemia. Systemic chemotherapy can improve the overall survival of patients.