儿童alk阳性的淋巴组织细胞型间变性大细胞淋巴瘤-第三军医大学学报.docVIP

儿童ALK阳性的淋巴组织细胞型间变性大细胞淋巴瘤 摘要 目的 探讨淋巴组织细胞型间变性大细胞淋巴瘤(ALCL-LH)的病理形态学特点、诊断。方法 通过光镜、免疫组化对2例ALCL-LH进行临床病理观察原位杂交检测EB病毒并结合文献加以分析。结果 2例ALCL-LH儿童患者临床表现为高热、淋巴结肿大伴肝脾肿大。形态学组织细胞和淋巴细胞较多，肿瘤细胞较少，可见围绕血管周围生长。免疫组化2例大部分瘤细胞CD30、ALK-1、EMA和表达，表达2例CD45、CD3、CD45RO、CD20、CD79a、CD15、MPO、CK、TdT、LMP1阴性原位杂交ERBR1/2。结论 ALCL-LH是少见类型，好发于，形态学特征为反应性组织细胞和淋巴细胞数量超过并掩盖肿瘤细胞。免疫组化染色CD30、ALK、EMA和阳性表达对诊断和鉴别诊断有重要作用。 关键词 淋巴组织细胞型间变性大细胞淋巴瘤； 病理； 诊断； ALK 中图分类号 R733号Anaplastic Large Cell Lymphoma，ymphohistiocytic Variant：tudy of Two Pediatric Patients with ALK+ LI Dan, MI Can, LI Yuan-Yuan. （Department of Pathology, College of Basic Medicine, Chongqing Medical University, Chongqing 400016, China） Abstract： Objective To study the clinicopathological characteristics, diagnosis, differential diagnosis of anaplastic large cell lymphoma, lympho- histiocytic variant (ALCL-LH ). Methods Clinical manifestations, pathological features, immunohistochemistry were analyzed, and examined by in situ hybridization for Epstein-Barr virus(EBV) DNA. Follow up in 2 cases with review of the literatures. Results Two cases all occurred in children. The clinical manifestations of 2 cases presented with fever and enlarged lymph nodes as well as hepatomegaly and Splenomegaly. Microscopically, the neoplastic cells more often of medium to large size and often smaller than in the common type, with irregular nuclei, were sparsely intermixed with a large number of histiocytes and lymphocytes that exceed and mask the tumour cell population, the tumor cells may cluster around blood vessels, case 1 with evidence of rythrophagocytosis. Immunohistochemical stains showed that tumor cells of two cases were positive for CD30, ALK, EMA, Granzyme B, as well as case 1 for CD43, case 2 for CD7. but two cases negative for CD45, CD2, CD3, CD4, CD5, CD8, CD45RO, CD56, CD20, CD79a, CD15, MPO, CK, TdT, LMP1. In addition, the EBV DNA was not detected in the lymphoma by in situ hybridization. Case 1 died ten days and case 2 three months after pathological diagnos