Wang Tingting, Jia Ling, Liao Wenjun, Chen Liuqing, Chen Xixue, Xiong Ya, Hao Fei, Zhu Xuejun, Yang Xichuan, Wang Lin
Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu 610041, China.
E-mail: lkzwl @126.com.
Zhonghua Bing Li Xue Za Zhi. 2015 Feb;44(2):100-5.
To study the clinicopathologic features, immunophenotype and gene rearrangement of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL).
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.
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, CD10 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.
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.
研究原发性皮肤弥漫性大B细胞淋巴瘤，腿部型（PCLBCL）的临床病理特征、免疫表型及基因重排。
7例PCLBCL纳入本研究。进行临床病理分析、免疫组化染色及IgH和Igκ基因重排检测。
7例均为男性，中位年龄72岁。患者通常表现为多发紫色肿物、结节、丘疹及浸润性斑块。2例发病前有腿部外伤史，1例有蚊虫叮咬史。组织学上，肿瘤累及真皮及皮下组织，呈致密弥漫性浸润模式，由中心母细胞和/或免疫母细胞组成。免疫组化染色显示，7例（7/7）表达CD20，6例（6/6）表达bcl-2，4例（4/4）表达MUM-1，4例（4/5）表达CD79a，4例（4/5）表达PAX-5，4例（4/6）表达bcl-6。所有病例均不表达CD3ε、CD45RO、CD10及CD30。3例（3/6）检测到IgH基因重排条带，1例（1/5）检测到Igκ基因重排条带。7例中有6例死亡，其余1例44岁患者，随访22个月后仍存活。
PCLBCL罕见，主要累及老年男性患者。PCLBCL预后差、死亡率高，但年轻患者预后似乎较好。部分病例有外伤或蚊虫叮咬史。该病史与PCLBCL发病的关系需进一步评估。
