原发皮肤CD4^+小/中等大T细胞淋巴组织增生性疾病临床病理特征及预后分析

Analysis of clinicopathological characteristics and prognosis of primary cutaneous CD4^+small/medium T-cell lymphoproliferative disorder

目的探讨原发皮肤CD4^+小/中等大T细胞淋巴组织增生性疾病(primary cutaneous CD4^+small/medium T-cell lymphoproliferative disorder,CD4^+PCSM-TCLD)的临床病理特征及预后不良因素。方法收集6例CD4^+PCSM-TCLD,分析其临床特点、组织学形态、免疫表型、原位杂交、基因重排检测及预后,并复习相关文献。结果6例患者女性2例,男性4例,发病年龄19~53岁,中位年龄36.5岁,病程1~12个月,临床均表现为头颈部皮肤的单发结节/斑块,病灶最大径10 cm。组织病理学形态相似,6例病变主要位于真皮层,均累及皮下脂肪组织,但表皮均未见受累。病变呈结节状或弥漫性生长模式,由大量致密的中小淋巴细胞组成,背景见多形性炎细胞浸润。上皮脚下延和肉芽肿结构各在1例中可见。免疫表型:CD2、CD4均阳性,CD5(3/6)阳性,CD3、CD7(5/6)阳性,滤泡辅助T细胞表型CD10(5/5)均阴性,Ki-67增殖指数最高达80%。6例EBER原位杂交检测结果均阴性,TCR基因重排检测均为单克隆表达。4例行单纯手术切除,1例手术后进行4个周期的化疗,1例失访。有随访资料的5例患者均无瘤生存,随访时间1~52个月。结论CD4+PCSM-TCLD是原发皮肤的少见淋巴组织增生性疾病,具有独特的临床病理特征,其形态学表现复杂多变,需与其它原发皮肤的淋巴瘤进行鉴别。

Purpose To investigate the clinicopathological features and poor prognostic factors of primary cutaneous CD4+small/medium T-cell lymphoproliferative disorder(CD4^+PCSM-TCLD).Methods 6 cases CD4^+PCSM-TCLD were collected,summarized their clinical features and pathological morphology,analyzed their immunohistochemical,in situ hybridization,gene rearrangement test results and prognosis,with review of relevant literature.Results There were 2 females and 4 males.The age of onset was 19 to 53 years,with median 36.5 years.Course of disease was 1-12 months.Clinical manifestations included single nodules/plaques on the skin of the head and neck,and the maximum diameter of the lesion was 10 cm.The histopathological morphology was similar.The lesions were mainly located in the dermis,and all of the 6 cases involved subcutaneous adipose tissue,but the epidermis was not affected.The lesions were in a nodular or diffuse growth pattern,composed of a large number of dense small/medium lymphocytes,and infiltrated with background polymorphic inflammatory cells.Epithelial foot extension and granuloma structure were seen in 1 case.All cases were positive for CD2 and CD4,positive for CD5(3/6),CD3(5/6)and CD7(5/6),follicular helper T cell phenotype CD10 was negative(5/5),Ki-67 proliferation index up to 80%.EBER in situ hybridization was negative in 6 cases and the TCR gene rearrangement was monoclonal expression in all the cases.4 patients underwent simple surgical resection,1 patient underwent 4 cycles of chemotherapy,and 1 patient was lost to follow-up.All 5 patients with follow-up data survived without tumor,and the follow-up time ranged from 1 to 52 months.Conclusion CD4^+PCSM-TCLD is a rare lymphoproliferative disease of primary skin.It has unique clinicopathological characteristics.Its morphological manifestations are complex and changeable,and it needs to be distinguished from other primary skin lymphomas.