摘要目的 分析T淋巴母细胞淋巴瘤合并朗格汉斯细胞组织细胞增生症(T-LBL合并LCH)的病理组织学、免疫表型和分子遗传学特征,探讨其诊断、鉴别诊断及预后相关因素.方法 收集3例T-LBL合并LCH的临床病理资料,采用HE、免疫组织化学(EnVision法)进行染色并分析,聚合酶链反应(PCR)技术检测肿瘤T细胞受体(TCR)基因重排情况.结果 3例患者均为男性,平均年龄61.7岁.镜下淋巴滤泡套区周围及副皮质区常见中等大小形态相对单一的T-LBL的肿瘤细胞,细胞胞质少,染色质细,偶见核仁,可见较多核分裂象及凋亡小体;部分区围绕套区呈单行列兵样排列.淋巴结被膜下及副皮质区常见成巢胞质丰富淡染、有明显核沟的朗格汉斯细胞增生;肿瘤或以两种肿瘤细胞相互穿插、或以两种肿瘤成分完全分离的方式分布,但3例均可见两种肿瘤细胞混合存在的区域;免疫组织化学示组织细胞表达:CD1a、S-100蛋白、CD68;淋巴母细胞表达CD3、CD7、末端脱氧核苷酸转移酶(TdT)和CD34.PCR技术检测到1例有TCR-γ基因重排.2例骨髓未累及,1例骨髓呈双表型急性白血病改变.3例患者均带瘤存活.结论 T-LBL合并LCH具有典型形态学、免疫表型和分子遗传学改变,应注意与皮病性淋巴结炎引起的郎格汉斯细胞组织细胞增生相鉴别,同时当临床出现LCH仅累及淋巴结时,需广泛取材,辅以免疫组织化学染色,排除有无合并淋巴瘤可能,尤其是误将高侵袭性淋巴瘤漏诊而延误患者治疗.T-LBL合并LCH两种肿瘤具有一定克隆相关性.

abstractsObjective To study the clinicopathologic features,immunophenotype and molecular genetic changes of T lymphoblastic lymphoma (T-LBL) associated with Langerhans cell histiocytosis (LCH).Methods Three cases of T-LBL associated with LCH were included.The morphologic characteristics were reviewed along with immunohistochemical profiling using EnVision method and TCR gene rearrangement by PCR.A review of composite lymphoma previously reported in the literature was performed.Results All three patients were male with the mean age of 61.7 years.One was Hans and the other 2 were Uyguers.All presented with superficial lymph node enlargement.Biopsy of lymph node showed two abnormal cell populations:distended sinus by large,pale histiocytes with nuclear grooves,and the interfollicular region containing immature-appearing cells with irregular nuclei slightly larger than that of small lymphocyte,dispersed chromatin,inconspicuous nucleoli,scant cytoplasm,and scattered mitotic figures.These cells presented in aggregates and small sheets interspersed with normal-appearing lymphocyte.The histiocytes were positive for CD1a,S-100 protein and CD68.The lymphoma cells were positive for CD3,CD7,TdT and CD34.TCR-γ gene rearrangement was detected in one case by PCR technology.One case involved bone marrow with double phenotype acute leukemia.Amongst the 8 including 5 reported cases,there were 4 males and 4 females.The mean age of the patients and the median age were 54 years.Lymphoadenopathy was the most common presentation.Bone marrow was involved in 4 cases.The time of follow-up was 2 to 27 months.The median survival was 5.5 months and the one-year survival rate was 33.3％.Conclusions Diagnosis of T-LBL and LCH should be based on typical morphology,immunophenotype and molecular genetic findings,with differential diagnoses including Langerhans cell hyperplasia originated from dermatopathic lymphadenopathy.When involving lymph node,extensive sampling supplemented by immunohistochemical staining is important to reach a correct diagnosis.Although coexistent T-LBL and LCH is clonally related,the understanding of its pathogenesis requires further investigation.