摘要目的 探讨原发性皮肤腿型弥漫性大B细胞淋巴瘤(PCLBCL)的临床病理学特点.方法 对7例PCLBCL患者的临床病理资料进行分析,并行免疫组织化学染色及IgH和Igκ基因重排检测.结果 7例均为男性,中位年龄72岁,临床表现为多发的紫红色肿块、结节、丘疹、浸润性斑块.2例有外伤史,1例有蚊虫叮咬史.组织学上肿瘤位于真皮及皮下脂肪层,常见无浸润带.瘤细胞呈弥漫性、密集浸润,2例伴有结节状浸润.浸润的瘤细胞主要为中心母细胞和/或免疫母细胞.7/7均表达CD20,6/6表达bcl-2,4/5表达CD79a,4/5表达PAX-5,4/4表达MUM-1,4/6表达bcl-6,瘤细胞均不表达CD10、CD30、CD3ε、CD45RO.3例(3/6)检出IgH基因克隆性重排条带,1例(1/5)检出Igκ基因克隆性重排条带.随访结果7例中6例死亡,1例44岁患者存活22个月.结论 PCLBCL罕见,好发于老年人,本组均为男性.该肿瘤进展迅速、预后差,年龄较小者预后较好.外伤或蚊虫叮咬与PCLBCL发病的相关性尚需更多的证据.

abstractsObjective To study the clinicopathologic features,immunophenotype and gene rearrangement of primary cutaneous diffuse large B-cell lymphoma,leg type (PCLBCL).Methods Seven cases of PCLBCL were enrolled into the study.Clinicopathologic analysis,immunohistochemical staining and gene rearrangement for IgH and Igκ were undertaken in the study.Results All the seven cases were male,and the median age was 72 years.Patients usually presented with multiple purple tumors,nodules,papules and infiltrative plaques.Two patients had a history of leg injury before onset,and one had mosquito bites.Histologically,the tumor involved the dermis and subcutis with dense and diffuse infiltrative pattern composing of centroblasts and/or immunoblasts.Immunohistochemical staining showed that seven cases (7/7) expressed CD20,six (6/6) expressed bcl-2,four (4/4) expressed MUM-1,four (4/5) expressed CD79a,four (4/5) expressed PAX-5 and four (4/6)expressed bcl-6,respectively.All cases did not express CD3ε,CD45RO,CD1O and CD30.IgH gene rearranged bands were detected in three (3/6)cases and Igκ was detected in one (1/5) case.Six of the seven cases died and the remaining patient,who was 44-year-old,was alive after 22 months of follow-up.Conclusions PCLBCL is rare,predominantly affects elderly male patients.PCLBCL has poor prognosis and high mortality,but younger patients seem to have better prognosis.Some cases had a history of trauma or mosquito bites.The relationship between the history and the onset of PCLBCL needs further evaluation.