【摘要】 目的观察原发皮肤CD4+小到中等大T细胞淋巴组织增生性疾病(Primary cutaneous CD4+small/medium T-cell lymphoproliferative disorder, PCSMTCLPD)临床病理特征。方法收集3例PCSMTCLPD患者临床资料,取病变组织进行病理检查,利用HE染色、免疫组化等方法明确诊断并随访,结合文献分析疾病的临床病理特点。结果 2例表现为背部皮肤单发性结节,1例表现为头颈部多发性结节;光镜下见真皮内致密淋巴细胞弥漫、结节状、条带状浸润,局部浸润到皮下脂肪组织,皮肤附件内可见少量淋巴细胞浸润,并见小血管增生现象;其中异型淋巴细胞形态小到中等大,形态多样,核深染,可见小核仁;免疫组化阳性表达CD3、CD4,部分阳性表达Bcl-6、PD-1、CXCL-13,阴性表达CD10、CD8,细胞增殖活性较低,Ki-67约10～20%,诊断为PCSMTCLPD。随访10～39月,未见病变复发或进展,患者健康状况良好。结论 PCSMTCLPD具有惰性临床过程,正确诊断并选择适当的治疗措施具有重要临床意义。更多还原

【Abstract】 Objective To describe the clinicopathologic features of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. Methods The clinical data and pathologic slides of three cases were retrospected. Hematoxylin and eosin-stained slides from formalin-fixed, paraffin-embedded tissue were reviewed, Immunohistochemical studies were performed on sections using an automated immunostainer. Results PCSMTCLPD presented as a single or multiple nodules located on the head, neck and the back; Microscopically, the lesions were characterized by a dense nodular, diffuse or band-like lymphoproliferation within the entire dermis extending sometimes into the subcutis, the pattern of epidermotropism, folliculotropism and capillary hyperplasia were present. Histopathological features of the lesions included predominance of small to medium-sized CD4+ T cells with occasional larger lymphoid forms and variable admixtures of CD8+ T cells, B cells, histiocytes, eosinophils and so on. The phenotype of atypical cells were immunopositive for CD3 and CD4, partial immunopositive for follicular T helper cells lineage markers of Bcl-6,PD-1,CXCL-13, immunonegative for CD8 and CD10. The proliferation rate(Ki67/MIB-1) in our cases was low, about 10～20%.The patients shared good health and no recurrence or progression during follow-up 10～39 months without any treatment. Conclusion PCSMTCLPD might present a peculiar indolent clinical course, It is important to be aware of these diseases for the correct diagnosis and optimal management.更多还原