原发皮肤CD4^(+)小到中等大T细胞淋巴组织增生性疾病临床病理观察

Clinicopathologic features of the primary cutaneous CD4+small/medium T-cell lymphoproliferative disorder

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。

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.