粒细胞肉瘤11例临床分析

Analysis of the clinical characteristics of granulocytic sarcoma from eleven cases

目的提高对粒细胞肉瘤(GS)的诊断、发病机制、治疗以及预后的认识。方法回顾性分析2003年2月至2008年5月河南省肿瘤医院收治的11例GS患者的误诊原因以及治疗方法对预后的影响。结果7例患者初诊时被误诊,分别被误诊为非霍奇金淋巴瘤2例,腰椎间盘突出症2例,乳腺肿瘤2例,神经母细胞瘤1例。误诊率为63.6%,其中形态学误诊4例(36.4%),医学影像学误诊3例(27.3%)。3例造血干细胞移植(HSCT)后复发者,既往均有AML-M2病史,同时有染色体t(8;21)异常,1例伴有del(11)(q)。8例死亡,中位生存时间20.5个月(6～40个月),其中5例接受常规治疗的患者中位生存期为10个月(6～23个月),3例接受大剂量阿糖胞苷化疗和(或)HSCT的患者中位生存期为35个月(32～40个月),明显优于常规治疗患者。结论GS在临床特征、医学影像学和形态学上容易误诊,免疫组织化学检查为其诊断的重要依据。联合骨髓细胞形态学和染色体核型分析可以帮助诊断。GS复发率高,预后差,高强度的化疗有望延缓复发,提高长期生存率。AML-M2伴有染色体t(8;21)可能为其易患因素。

Objective To enhance the awareness of diagnosis, pathogenesis, therapy, and prognosis of GS. Methods The retrospective analysis was used to analyze 11 cases of GS which were admitted and treated. Resuits 7 patients were misdiagnosed, two of which were misdiagnosed as non-Hodgkin lymphoma,two as prolepses of lumbar intervertebral dise, two as breast neoplasm and one as euroblastoma. The rate of misdiagnosis was 63.6%, with 4 of morphological misdiagnosis（36. 4% ）and 3 of medical imageology （27. 3% ）. In addition, three patients who relapsed after HSCT had a history of the AML-M2 and had the chromosome abnormality of t（8 ;21 ） and one was accompanied by the del （ 11 ） （q）. 8 patients died, with the median survival of 20. 5 months （6 -40）. The median survival of the five patients who accepted conventional chemotherapy was 10 months （ 6 -23 ）. The median survival of three patients who accepted high-dose cytarabine and （or） accepted hematopoietic stem cell transplantation was 35 months （32 - 40）, with a significant advantage to the patients who accepted the conventional chemotherapy. Conclusion GS is tend to be misdiagnosed for its chnical features, medical imageology and morphological characteristics. Combination of bone marrow of morphocytology and karyotype analysis of chromosome may assist diagnosis. Due to the high relapse rate of GS and the poor prognosis, high-intensity chemotherapy might be accounted on to postpone the relapse and improve long-term survival. AML-M2 with the chromosome abnormali- ty of t（ 8 ;21） might be a risk factor of GS.