【摘要】 目的报道1例皮肤血管内T细胞淋巴瘤(T-IVL)并结合相关文献分析其临床病理特征。方法通过光镜及免疫组化(EnVison法)检测CD3、CD5、CD20、CD34、CD79α,分析其临床病理特征及免疫组化特点。结果本例为男性患者,43岁。患有慢性乙型肝炎,临床表现为乏力、发热伴腰腹部红斑,红斑边界不清有融合,局部压痛,可触及基底有结节,未触及肿大淋巴结,骨髓涂片无特殊。皮肤活检镜下可见真皮血管腔内有多量异型的单核肿瘤细胞,免疫组化肿瘤细胞CD3(),CD5(+/-),CD20、CD79α和CD34(-);血管CD34(+)。EBER原位杂交(+)。确诊后采用CHOP方案联合化疗2个疗程,皮疹渐消,仍发热,化疗后1个月死亡。结论T-IVL为一种高度恶性的罕见的非霍奇金淋巴瘤,可累及全身各个系统,最常累及皮肤,病变多局限于血管腔内,病理检查及免疫组化、基因重排对确定诊断有重要意义。本病预后差,联合化疗有助于疾病治疗。更多还原

【Abstract】 Objective To report a case of intravascular T-cell lymphoma(T-IVL) of skin and to study the clinicopathologic features of IVL with review of the literature.Methods A case of T-IVL of skin was studied by light microscopy and immunohistochemical staining for CD3,CD5,CD20,CD34,and CD79α.Results The patient,a 43-year-old Chinese man with HBsAg +,HBeAb + and HBcAb +,had fever and reddish macules scattered over the two sides of lumbar and abdominal part surface,and neither lymphadenopathy nor hepatosplenomegaly was recognized.Skin biopsy showed large mononuclear cells within small-and mediun-sized blood vessels.Immunohistochemical study showed tumor cells revealed CD3+,CD5+/-,CD20-,CD34-,and CD79α-.EBER in situ hybridization was positive.The patient treated with combination chemotherapy and died at 1 month after diagnosis,and the whole course of disease was only 6 months.Conclusions T-IVL is a rare high-grade non-Hodgkin lymphoma characterized by massive proliferation of large cells within small and mediun-sized blood vessels,custaneous involvement is common and every organs can also be involved.Diagnosis of IVL requires pathologic examination,immunohistochemistry and gene rearrangement analysis.The prognosis is poor and remission and long-term survival may result from combination chemotherapy.更多还原