淋巴组织细胞型间变性大细胞淋巴瘤临床病理观察

Anaplastic large cell lymphoma, lymphohistiocytic variant:a report of three cases and review of literatures

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、诊断及鉴别诊断。方法通过光镜、免疫组化、EB病毒原位杂交对3例ALCL-LH进行临床病理观察,并结合文献加以分析。结果3例ALCL-LH患者均为儿童,临床表现为高热伴淋巴结肿大。形态学上组织细胞多,肿瘤细胞少,可见围绕血管周围生长。免疫组化3例均CD30、EMA和ALK(+),CD3、CD20(-),2例CD2(-)。原位杂交肿瘤细胞EB病毒(-)。结论ALCL-LH是一种少见类型,好发于青少年,形态学特征为多量反应性组织细胞和淋巴细胞,夹杂少量大细胞。免疫组化染色CD30、EMA和ALK(+)对诊断和鉴别诊断有重要作用。

Objective To study the clinicopathological characteristics, diagnosis, differential diagnosis of anaplastic large cell lymphoma, lymphohistiocytic variant （ ALCL-LH ）. Methods Clinical manifestations, pathological features, immunohistochemistry and Epstein-Barr virus （EBV） in situ hybridization were analyzed in 3 cases with review of the literatures. Results Three eases all occurred in children. The clinical manifestation of 3 cases presented with fever and enlarged lymph nodes. Microscopically, tumor cells may cluster around blood vessels. Immunohistochemical stains showed that tumor cells were positive for CD30, EMA, ALK, and negative for CD3 and CD20. CD2 was positive in 2 cases. EBV in siut hybridization showed tumor cells were negative. Conclusions ALCL-LH variant is a rare subtype, The tumors often occur in young patients. Microscopically, ALCL-LH is characterized by a few large cells admixed with a large number of histiocytes and lymphocytes. Immunohistochemically, the expression of CD30, EMA and ALK play an important role in diagnosis and differential diagnosis.