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目的探讨儿童淋巴组织细胞型间变性大细胞淋巴瘤(ALCL-LH)的临床病理特征、诊断及预后。方法通过光镜、免疫组化对2例ALCL-LH进行临床病理观察及原位杂交检测EB病毒,并结合文献加以分析。结果2例ALCL-LH儿童患者临床主要表现为高热、淋巴结肿大伴肝脾肿大。形态学示淋巴结结构大部分破坏,背景中组织细胞、淋巴细胞和小血管增生较多,肿瘤细胞较少,细胞中等偏大,但比普通型小,核型不规则,可见围绕血管周围生长。免疫组化示2例大部分瘤细胞CD30、ALK-1、EMA和GranzymeB阳性表达,例1CD43、例2CD7阳性表达,2例CD45、CD2、CD3、CD4、CD5、CD8、CD45RO、CD56、CD20、CD79a、CD15、MPO、CK、TdT、LMP1阴性,原位杂交EBER1/2阴性。随访2例均死亡,分别存活10d及3个月。结论ALCL-LH是恶性淋巴瘤的极少见类型,好发于儿童,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和细胞毒性颗粒相关蛋白(如GranzymeB等)阳性表达对诊断和鉴别诊断有重要作用。
Objective To investigate the clinicopathological features, diagnosis and prognosis of childhood lymphoid cell metamerising large cell lymphoma (ALCL-LH). Methods Two cases of ALCL-LH were observed by light microscopy and immunohistochemistry. The EBV was detected by in situ hybridization and analyzed by literature. Results The clinical manifestations of 2 ALCL-LH children were high fever, swollen lymph nodes and hepatosplenomegaly. Morphology showed most of the destruction of lymph node structure, the background of tissue cells, lymphocytes and small blood vessels more proliferation, fewer tumor cells, cells larger than normal, but smaller than the normal type, karyotype irregular, visible around the perivascular growth. Immunohistochemistry showed that the positive expression of CD30, ALK-1, EMA and GranzymeB were found in most of the tumor cells in two cases, including 1 CD43 and 2 CD7 positive in 2 cases, 2 cases CD45, CD2, CD3, CD4, CD5, CD8, CD45RO, CD56, CD20, CD79a, CD15, MPO, CK, TdT, LMP1 negative, in situ hybridization EBER1 / 2 negative. Two patients were followed up for 10 days and 3 months respectively. Conclusions ALCL-LH is a rare type of malignant lymphoma that occurs in children with morphologic features of over-expressing reactive cells and lymphocytes and masking tumor cells. Immunohistochemical staining of CD30, ALK, EMA and cytotoxic particle-associated protein (such as GranzymeB) positive expression of the diagnosis and differential diagnosis plays an important role.