T淋巴母细胞性淋巴瘤/髓系肉瘤合并朗格汉斯细胞组织细胞增生症的临床病理分析

目的探讨T淋巴母细胞性淋巴瘤(T-LBL)/髓系肉瘤(MS)合并朗格汉斯细胞组织细胞增生症(LCH)的临床病理学特征、免疫表型及预后。方法收集中山大学附属佛山医院和首都医科大学北京友谊医院2013年12月至2019年4月间6例T-LBL/MS合并LCH患者临床及病理资料,采用HE染色、免疫组织化学EnVision法、原位杂交法进行染色分析,检索文献并复习。结果6例患者中,男性2例,女性4例。4例为T-LBL合并LCH,1例为T-LBL/MS合并LCH,1例为MS合并LCH。患者年龄5~77岁,中位年龄59岁。3例为多发淋巴结内病变,另3例为多发淋巴结及皮肤/肝脾病变。5例显示淋巴结结构破坏,3例可见数个残留萎缩的滤泡。瘤细胞有2种形态,一种为体积中等大小的淋巴样细胞,呈片巢状分布。核圆形、卵圆形,这类细胞主要分布于残留的滤泡旁和副皮质区。另一种为组织细胞样细胞,体积大,胞质丰富淡染或嗜双色性。核卵圆形、不规则形,呈折叠状,主要分布于淋巴结边缘窦、髓窦和滤泡间区。所有病例的背景嗜酸性粒细胞浸润不明显。中等大小淋巴样细胞表现为末端脱氧核苷酸转移酶(TdT)、CD99、CD7阳性,不同程度表达CD34、髓过氧化物酶(MPO)、CD2、CD3,Ki-67阳性指数多在30%~50%之间。而组织细胞样细胞表现为CD1a阳性、S-100蛋白阳性、Langerin表达不一,不同程度表达CD163/CD68,不表达T和B细胞标志物,Ki-67阳性指数多在10%~20%之间。所有病例均无EB病毒感染。随访4例(随访时间6~63个月,中位时间18.5个月),其中1例死亡,3例带病生存。结论T-LBL/MS合并LCH是一种少见的混合型幼稚淋巴造血系统疾病,多发生于皮肤、淋巴结,临床进展凶猛,预后差,故充分认识同一组织内两种病变成分有助于精准诊断与治疗。

Clinicopathological features of T-lymphoblastic lymphoma with Langerhans cell histiocytosis in the same lymph node

Objective To investigate the clinicopathological features,immunophenotypes,genetics and prognosis of T-lymphocyte lymphoma/myeloid sarcoma combined with Langerhans cell histiocytyosis(coexistence of T-LBL/MS and LCH).Methods Clinical and pathological data of the 6 patients with coexistence of T-LBL/MS and LCH were analyzed,who were diagnosed at the Foshan Hospital of Sun Yat-sen University and the Friendship Hospital of Capital Medical University,from December 2013 to April 2019.The hematoxylin and eosin stain,immunohitochemistry(EnVision)and in situ hybridization were used.Related literatures were reviewed.Results Four patients were T-LBL combined with LCH,1 was T-LBL/MS combined with LCH,and 1 was MS combined with LCH.There were 2 male and 4 female patients,with age ranged from 5 to 77 years old(median,59 years old).Three patients represented with only multiple lymph node swelling.The other 3 displayed both multiple lymph node swelling,and skin/liver or spleen lesions.Lymph node structure was destroyed in 5 cases,while 3 cases had several residual atrophic follicles.Histologically,there were two types of tumor cells:one type of the abnormal lymphoid-cells exhibited small to medium-sized blast cells,typically showing a nested distribution,and these cells were mainly identified in residual follicles and paracortical areas;the other type of histiocytoid cells had a large cell size and abundant pale or dichromatic cytoplasm.Their nuclei were irregularly shaped,showing folded appearance and nuclear grooves.These cells were mainly present in marginal sinus,medullary sinus and interstitial area between follicles.Eosinophil infiltration in the background was not evident in any of the cases.The lymphoid-cells of medium size showed TdT+/CD99+/CD7+,with variable expression of CD34/MPO/CD2/CD3.Ki-67 index was mostly 30%-50%.However,the histiocytoid cells showed phenotype of CD1a+/S-100+/Langerin+/-,while CD163/CD68 were positive in some degree.These cells did not express any T or B cell markers.The Ki-67 index mostly ranged between 10%-20%.None of the cases had Epstin-Barr viral infection.Among the 6 patients,4 patients were followed up(6-63 months,median time,18.5 months),of whom 1 patient died of the disease and 3 patients were alive at the end of follow-up.Conclusions T-LBL/MS combined with LCH is a rare mixed type of immature hematopoietic disease,and mainly occurs in lymph node and skin.The clinical course is overall aggressive.Therefore,it is helpful to recognize and identify the two pathologic components in the same tissue for accurate diagnosis and proper treatment.