原发性皮肤γδT细胞淋巴瘤的临床病理研究

Clinicopathologic study of primary cutaneous gamma delta T-cell lymphoma

目的探讨原发性皮肤γδT细胞淋巴瘤(PCGD-TCL)的临床病理特点。方法回顾性分析2007~2018年间首都医科大学附属北京友谊医院病理科诊断的11例PCGD-TCL患者的临床病理特征、免疫组化结果,并复习相关文献。结果 11例PCGD-TCL患者中,男性7例,女性4例;年龄为13~63岁,平均35.8岁。临床表现为皮肤浸润性斑块、皮下结节或肿块,其中6例伴有溃疡形成。11个病例中,8例显示肿瘤细胞浸润真皮及皮下组织,2例显示肿瘤细胞仅浸润真皮,1例显示肿瘤细胞浸润表皮及真皮。瘤细胞中等大小,染色质粗块状。免疫组化结果显示,阳性表达情况为CD2(5/7)、CD3(11/11)、CD5(3/10)、CD7(4/9)、TIA-1(9/9)、粒酶B(9/9)、TCRαβ(0/11)、TCRγδ(11/11)、CD56(10/11)。11例行EB病毒原位杂交检测均为阴性。结论 PCGD-TCL是一种非常少见的皮肤T细胞淋巴瘤,确诊需要依靠病理形态学及免疫组织化学,其中TCRγδ的表达更具有诊断意义。

Objective To investigate the clinicopathologic features of primary cutaneous gamma delta T-cell lymphoma (PCGD-TCL).Methods The clinicopathologic features and immunophenotypic findings of 11 cases of PCGD-TCL diagnosed in the department of pathology,Beijing Friendship Hospital,Capital Medical University from 2007 to 2018 were retrospectively analyzed,and the related literatures were reviewed.Results Among the 11 PCGD-TCL patients,7 were male and 4 were female,with an average of 35.8 years old (ranged:13~63 years old).All patients presented with skin infiltrate plaque,subcutaneous nodules or masses,of which 6 cases were accompanied by ulceration.Of the 11 cases in this group,8 showed tumor cells infiltrating the dermis and subcutaneous tissues,2 showed tumor cells infiltrating only the dermis,and 1showed tumor cells infiltrating the epidermis and dermis.The neoplastic cells were medium in size,with coarsely clumped chromatin.The immunohistochemical staining results were positive for CD2 (5/7),CD3 (11/11),CD5 (3/10),CD7 (4/9),TIA-1 (9/9),Granzyme-B (9/9),TCRαβ(0/11),TCRγδ(11/11),CD56 (10/11).EB virus infection was not detected in all patients by in situ hybridization.ConclusionPCGD-TCL is a rare form of cutaneous T-cell lymphoma.Its diagnosis depends on pathomorphological examination and immunophenotyping,and expression of TCRγδis of diagnostic significance.