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Extramedullary acute biphenotypic acute leukemia/lymphoma: a clinicopathologic and immunophentypic study
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- Author:
- No author available
- Journal Title:
- JOURNAL OF LEUKEMIA & LYMPHOMA
- Issue:
- 5
- DOI:
- 10.3760/cma.j.issn.1009-9921.2007.05.004
- Key Word:
- 双表型白血病/淋巴瘤;髓外浸润;免疫表型分型
Abstract： 目的 进一步明确髓外双表型急性白血病/淋巴瘤的临床病理特点、了解免疫表型检测在其诊断和鉴别诊断中的作用,为临床规范化治疗提供科学的信息.方法 选用北京大学基础医学院病理学系淋巴瘤研究室2004年9月至2006年12月诊断为髓外双表型白血病/淋巴瘤的病例共9例,所有患者均结合细胞形态学、免疫表型检测并参照急性白血病免疫学特征欧洲协作组(European group for the immunologocal cal characterization of acute leukemias,EGIL)评分系统诊断.免疫组织化学染色采用DAKO公司EnVisionTM法进行髓系、淋巴细胞系多种标记物的检测.并结合临床资料进行临床病理分析.结果 髓外双表型白血病/淋巴瘤主要发生在青少年,中位年龄是13岁,其中6例的年龄＜18岁.男女之比为1.25:1.病变的部位分布以淋巴结最为常见(7例),其次为皮肤(2例).免疫表型检测显示8例为髓系/T淋巴系(myeloid/T-lymphoid,M/T)双表型,1例为B/T淋巴系(myeloid/B-lymphoid,M/B)双表型.随访7例,时间为2～18个月,其中6例经临床检查骨髓和外周血,证实存在白血病;另1例目前尚未出现骨髓异常.2例在确诊后2个月死亡,其余目前一般情况尚可.结论 髓外双表型急性白血病/淋巴瘤发病率低,可伴随白血病出现,也可孤立发生.免疫表型以M/T双表型多见.免疫组织化学检测是诊断的重要手段.全面进行多项标记物的检测,可大大减少漏诊和误诊,为临床的准确诊断和及时治疗提供有力的依据.
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