小细胞性外周T细胞淋巴瘤的病理特征及临床意义

Small cell variant of periphral T-cell lymphoma the pathological signs and clinicopathologic analysis

目的:探讨非特殊性外周T细胞淋巴瘤(PTCL,NOS)的病理特征。方法:对5例小细胞性PTCL,NOS进行临床病理回顾性分析和随访,免疫组化分析采用SP法。组织学分类根据2008年世界卫生组织关于淋巴造血组织分类标准。结果:5例患者中男4例,女1例;年龄45～77岁,平均58.4岁。临床1期4例,病灶局限于局部淋巴结肿大、鼻咽部斑块、结肠肿块。临床3期1例,有脾肿大,骨髓肿瘤累及,外周血淋巴细胞升高。肿瘤增生的淋巴结结构破坏,为单一的小淋巴细胞取代。图像中见有少量大细胞混杂3例,同时伴有小血管增生2例。免疫组化标记肿瘤细胞有2种以上T细胞标记表达、CD43阳性、CD20和CD46阴性。有4例CD99阳性。随访:死亡1例,存活2例,失访2例。结论:小细胞性PTCL,NOS少见,组织学形态表现为惰性淋巴瘤,当本病进入高临床分期时预后差。

Objective：To study the clinicopathologic features and differential diagnosis of small cell variant of periphral Tcell lymphoma,not otherwise specified （PTCL,NOS）.Methods：The clinicopathologic features of 5 cases of small cell variant of PTCL,NOS were retrospectively reviewed,with immunohistochemical study.Results：The 4 patients were males,one patients was femal.All the patients were older.Clinically,4 patients presented in stage Ⅰ and one in stage Ⅲ.Marrow involvement was detected in one patient with bonemarrow biopsy performed and he is also accompanied by lymphocytosis.Histologically,the involved lymph nodes showed partial or complete effacement of nodal architecture and replacement by a monomorphous population of small lymphoid cells.Scanty large lymphoid cells were also identified in 3 lymph node cases.Increase in number of blood vessels was noticed in two of them as well.Immunohistochemically the lymphoma cells in all cases expressed two or more of the T-cell markers and CD43,CD99 expression was noted in 4 of the 5 cases.The staining for CD20 and CD56 was negative.Follow up information was available in 5 cases.One of the patient died of the disease,the others were alive more than 2 years.Conclusion：Small cell variant of PTCL,NOS was a rare disease with histologically mature small cell feature.It carries a poor prognosis when the disease presents in advanced tumor stage.