淋巴瘤样丘疹病临床病理分析

Investigation on clinicopathologic features of lymphomatoid papulosis

目的探讨淋巴瘤样丘疹病（LyP）的临床病理特征、免疫表型及预后。方法分析13例LyP的临床病理资料，用LSAB法、Eli Vision法行T淋巴细胞、B淋巴细胞、活化淋巴细胞和细胞毒性等12种免疫组织化学标记，原位杂交检测EB病毒。结果13例LyP中男6例，女7例，平均年龄26．4岁，皮损多表现为四肢、躯干无症状的多发性丘疹、结节。组织学分型：A型6例，B型1例，C型6例，主要在真皮与皮下脂肪组织内浸涧，主要浸润方式分别为楔形、带状及片状、结节状。13例中8例有瘤细胞亲表皮现象；1例B型无大瘤细胞，余12例的大瘤细胞均表达CDS0。13例瘤细胞均表达2～3个T细胞标记（CD3、CD5或CIM5RO）及1～3个细胞毒性标记[T细胞胞质内抗原（11A）-1、粒酶B或穿孔素]；13例均表达CIM、4例表达CD8、1例表达CD15、1例表达CD20（局灶阳性），均不表达间变性淋巴瘤激酶（ALK）-1。亲表皮之瘤细胞多为CD3^＋、CD4^＋、CD8^＋表型。13例中1例EBER1／2原位杂交阳性。获随访的12例均存活。结论LyP有独特的临床病理表现和免疫表型，预后较好。A型和C型中亲表皮的瘤细胞均为核扭曲、深染，似覃样霉菌病细胞，推测其与CD30^＋大瘤细胞可能来源于不同的克隆群体有关。EB病毒与LyP可能无明确的相关性。

Objective To investigate the clinicopathologic features, immunophenotype and prognosis of lymphomatoid papulosis （LyP）. Methods Clinicopathologic analysis, immunohistoehemical staining （LSAB and EliVision method） and in situ hybridization for EBER were undertaken in this study. Results Thirteen cases of LyP were studied, derived from six male and seven female patients with a median age of 26.4 years. The most common presentation was multiple symptomless papules or nodules, involving predominately the extremities and trunks. Histologically, the tumor primarily involved the dermis and subcutaneous layer. Six tumors were type A, one was type B and six were type C. The main infiltration patterns were wedge-shaped, band-like, sheet-like or nodular. There was epidermotropism in eight cases. Immunohistochemical staining showed that the large tumor cells in all 12 types A and C cases expressed CD30. All 13 cases expressed two to three T-cell associated antigens （CD3, CD5 or CD45RO） and one to three cytotoxie granule associated antigens （TIA-1, GrB or Perforin）. All cases expressed CD4, four expressed CD8, and one expressed CD15. Only one case expressed CD20; and all cases were negative for ALK-1. The tumor cells showing epidermotropism had CD3^＋ , CD4^＋ and CD8- phenotype in most cases. Only one case was EBER1/2 positive. Follow up information was available in 12 patients; all were alive at the end of the follow up period. Conclusions LyP has distinctive clinicopathologic features and immunophenotype with favorable prognosis. In types A and C, the atypical ceils showing epidermotropism were similar to those in MF, these cells possess cerebriform and hyperchromatic nuclei. The epidermotropic tumor cells and the CD30^＋ large cells may be derived from different clones. EB virus may not be correlated with LyP.