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目的探讨粒细胞肉瘤的发病机制、诊治与转归。方法分析1例以CD56+小肠粒细胞肉瘤为首发表现的急性单核细胞白血病患者的临床病理特征及实验室检查结果,并复习相关文献。结果收治1例男性患者,39岁。小肠系膜切除物免疫组化检测示:粒细胞肉瘤,CD56阳性;骨髓细胞学检查示:急性单核细胞白血病,诊断为急性单核细胞白血病伴小肠粒细胞肉瘤。经IA方案诱导化疗后疾病达完全缓解,继续IA方案巩固化疗,拟行异基因造血干细胞移植。结论以小肠粒细胞肉瘤为首发表现的急性白血病较为少见。粒细胞肉瘤的预后差,CD56抗原阳性提示预后不良,经诱导化疗获疾病缓解的患者应尽快行异基因造血干细胞移植以期达到长期生存。
Objective To investigate the pathogenesis, diagnosis, treatment and prognosis of granulocystic sarcoma. Methods One case of acute monocytic leukemia with CD56 + small granulocytic sarcoma was analyzed for clinical and pathological features and laboratory findings. The related literatures were reviewed. Results One male patient was admitted, 39 years old. Small intestinal mesenteric excision immunohistochemistry showed: granulocytic sarcoma, CD56 positive; bone marrow cytology showed: acute monocytic leukemia, the diagnosis of acute monocytic leukemia with small intestinal granulocytic sarcoma. After induction of chemotherapy by IA regimen complete remission of disease, continued to strengthen the chemotherapy regimen IA, to be allogeneic hematopoietic stem cell transplantation. Conclusions Small-cell granulocytic sarcoma is the first manifestation of acute leukemia is rare. The poor prognosis of granulocytic sarcoma, positive CD56 antigen prompted a poor prognosis, chemotherapy-induced disease patients should be as soon as possible allogeneic hematopoietic stem cell transplantation in order to achieve long-term survival.