髓系肉瘤12例临床特点分析

Clinical characteristics of myeloid sarcoma: analyses of 12 cases

目的 探讨髓系肉瘤(MS)患者临床特点、疗效及预后.方法 回顾性分析2008年8月至2018年5月于空军军医大学西京医院确诊的12例MS患者临床资料,对其临床表现、诊治及生存情况进行分析.结果 12例患者年龄17~62岁,首发部位包括淋巴结区、外耳道、眼、臀、肺、肝、胰、乳腺、皮肤、椎体及其周围、子宫颈;经外周血分类、骨髓穿刺及骨髓活组织检查的11例患者中,孤立性MS 6例[1例进展为急性髓系白血病(AML)],MS伴慢性粒细胞白血病(CML)慢性期1例,MS伴AML-M22例,MS伴骨髓增生异常综合征(MDS)1例,再生障碍性贫血(AA)后MS 1例(骨髓无浸润).免疫组织化学结果显示,LCA(+)(7/7),MPO(+)(12/12),CD43(+)(9/9),lysozyme(+)(5/7),CD3(-)(8/8),CD20(-)(9/9),CD34(+)(5/6),CD117(+)(7/7),Ki-67(+)30%~90%.4例患者行骨髓染色体检测,2例伴AML患者的染色体为t(8;21),1例伴MDS患者为47、XX、+8、del(11)(q21),1例伴CML患者为t(9;22).12例患者中2例失访.随访的10例患者中6例死亡,4例生存,中位生存时间21个月(2~27个月).结论 AA稳定期伴MS、CML慢性期伴MS鲜见报道.MS患者临床表现多样,常见发病部位为浅表淋巴结,少见部位为椎体及其周围,罕见部位为眼部、胰腺、肺部、肝脏等.患者中位生存期短,疗效差.

Objective To investigate the clinical characteristics, curative effect and prognosis of myeloid sarcoma. Methods The clinical data of 12 patients with MS diagnosed at Xijing Hospital of Air Force Medical University from August 2008 to May 2018 were retrospectively analyzed. Their clinical manifestations, diagnosis, treatment and survival were analyzed. Results Twelve patients were 17 to 62 years old. The initial site included lymph node, external auditory canal, eye, buttock, lung, liver, pancreas, breast,skin, vertebra and its surroundings,and cervix. Among 11 patients with peripheral blood classification, bone marrow aspiration and bone marrow biopsy, 6 cases were isolated MS [one of which developed acute myeloid leukemia (AML)], 1 case was chronic myeloid leukemia in chronic phase, 2 cases were AML-M2, and 1 case was myelodysplastic syndrome (MDS), and 1 case was after aplastic anemia (AA) with no infiltration of bone marrow. Immunohistochemical results showed that LCA(+) (7/7), MPO(+) (12/12), CD43(+) (9/9), lysozyme(+) (5/7), CD3(-) (8/8), CD20(-) (9/9), CD34(+) (5/6), CD117(+) (7/7), and Ki-67(+) 30%-90%. Four patients were examined for bone marrow chromosomes, 2 patients with AML had t (8;21), 1 patient with MDS was 47, XX, +8, del(11)(q21), and 1 patient with CML was t(9;22). Two of the 12 patients were lost to follow-up. Among the 10 patients who were followed up, 6 died and 4 survived, and the median survival time was 21 months (2-27 months). Conclusions AA in stable phase with MS and CML in chronic phase with MS are rarely reported. The clinical manifestations of MS patients are varied, of which the common incidence sites are superficial lymph nodes, the infrequent sites are vertebra and its surrounding areas, and the rare sites are eye, pancreas, lung, liver, etc. The median survival time of MS patient is short and the curative effect is poor.