CAJ | 학술논문

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.
목적 분석T림파모세포림파류합병랑격한사세포조직세포증생증(T-LBL합병LCH)적병리조직학、면역표형화분자유전학특정,탐토기진단、감별진단급예후상관인소.방법 수집3례T-LBL합병LCH적림상병리자료,채용HE、면역조직화학(EnVision법)진행염색병분석,취합매련반응(PCR)기술검측종류T세포수체(TCR)기인중배정황.결과 3례환자균위남성,평균년령61.7세.경하림파려포투구주위급부피질구상견중등대소형태상대단일적T-LBL적종류세포,세포포질소,염색질세,우견핵인,가견교다핵분렬상급조망소체;부분구위요투구정단행렬병양배렬.림파결피막하급부피질구상견성소포질봉부담염、유명현핵구적랑격한사세포증생;종류혹이량충종류세포상호천삽、혹이량충종류성분완전분리적방식분포,단3례균가견량충종류세포혼합존재적구역;면역조직화학시조직세포표체:CD1a、S-100단백、CD68;림파모세포표체CD3、CD7、말단탈양핵감산전이매(TdT)화CD34.PCR기술검측도1례유TCR-γ기인중배.2례골수미루급,1례골수정쌍표형급성백혈병개변.3례환자균대류존활.결론 T-LBL합병LCH구유전형형태학、면역표형화분자유전학개변,응주의여피병성림파결염인기적랑격한사세포조직세포증생상감별,동시당림상출현LCH부루급림파결시,수엄범취재,보이면역조직화학염색,배제유무합병림파류가능,우기시오장고침습성림파류루진이연오환자치료.T-LBL합병LCH량충종류구유일정극륭상관성.
Objective 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.