T-淋巴母细胞淋巴瘤合并朗格汉斯细胞组织细胞增生症临床病理观察

目的 探讨T-淋巴母细胞淋巴瘤合并朗格汉斯细胞组织细胞增生症的病理特征及发病机制.方法 筛选广东省人民医院2002-2010间T-淋巴母细胞淋巴瘤病例组织切片,查找其伴发朗格汉斯细胞组织细胞增生症的病例.进行免疫组化标记及基因重排检测并随访.结果 筛选出病例2例,男女各1例,平均年龄20岁.均为无诱因出现浅表多发淋巴结肿大.镜下均可见两种细胞即淋巴母细胞性淋巴瘤细胞和朗格汉斯细胞.免疫组化前者CD3、TdT和CD7(+)；后者CD1a和S-100(+).基因重排结果为TCR-γ基因未见重排.2例分别于诊断后7个月和8个月时死亡.结论 T-淋巴母细胞淋巴瘤合并朗格汉斯细胞组织细胞增生症的现象非偶然现象.患者均表现为浅表淋巴结肿大,有或无B症状,预后差.病因考虑为:①TCR-γ基因重排结果阴性支持T淋巴母细胞分化处于胸腺前阶段,可能具有前体细胞的作用,具备分化成其他细胞系的能力.②为朗格汉斯细胞介导对恶性淋巴瘤的免疫反应,而非真正的瘤性病变.③两种肿瘤克隆相关.具体发病机制目前尚不明确,仍需进一步研究.

Simultaneous occurrence of T-lymphoblastic lymphoma and Langerhans cell histiocytosis in the same lymph node

Objective To report two cases of precursor T-lymphoblastic leukemia/lymphoma(T-LBL) coexistent with Langerhans cell histiocytosis(LCH) in the same lymph nodes,to discuss the clinicopathologic features and pathogenesis of this phenomenon.Methods All the T-LBL between 2002 and 2010 were reviewed to look for cases coexistent with LCH.Immunohistochemistry and gene rearrangement detections were done.The prognosis was followed up.Results Here we presented 2 patients(1 male and 1 female).The mean age was 20.0 years.Both were with history of multiple enlarged peripheral lymph nodes and no B symptoms.Histological findings showed two different cell populations as LBL cells and langerhans cells.CD3,TdT and CD7 were positive in the former,and CD1a and S100 were positive in the latter.No TCR-γ generearrangement was found.The women and the man died 8 and 7 months after diagnosis,respectively.Conclusion Simultaneous occurrence of T-LBL and LCH is not incidental.The patients show multiple enlarged peripheral lymph nodes and a poor prognosis.About the etiopathogenisis,it has a few arguments.1.Lymphoblastic cells lack rearrangements of TCR-γ genes,which suggests that LBL cells could act as progenitor cells with the ability to differentiate into other lineaages.2.LCH represents a reaction to the coexisting malignant lymphoma rather than a true neoplastic process.3.The two tumors are clonally related.However,the etiopathogenisis is still unclear and needs a further investigation.