摘要
患者男,90岁,全身红皮病,伴顽固性瘙痒和脱屑3+年,淋巴结肿大和白细胞升高1+月。外周血淋巴细胞数达41.83×109/L。外周血流式细胞免疫荧光分析结果:T淋巴细胞占有核细胞总数约为56.5%,免疫表型为CD2(+),CD3(+),CD5(+),CD4(+),CD8(-),CD7(表型丢失),CD4∶CD8>10。TCRγ基因重排检测(片段分析)检测到单克隆重排。骨髓穿刺活检组织病理检查示:间质内见散在脑回状细胞(Sézary细胞)浸润,免疫表型为CD2(弱+),CD3(弱+),CD4(弱+),CD8(-),CD5(弱+),CD7(表型丢失),CD43(弱+),Ki-67增殖指数约35%。病理诊断为Sézary综合征累及骨髓。
A 90-year-old male patient presented with generalized erythrodermas with intractable itching and scaling for 3＋ years,and lymphadenopathy and increased WBC for 1＋ month. Peripheral blood lymphocyte count was 41.83×109/L. Flow cytometry immunofluorescence of peripheral blood showed that T lymphocytes accounted for 56.5% of total nucleated cells,and their immunophenotypes were CD2（＋）,CD3（＋）,CD5（＋）,CD4（＋）,CD8（-）,loss of immunophenotype of CD7,and CD4：CD8〉10. TCR gamma rearrangement detection（fragment analysis）exhibited a monoclonal rearrangement. Histopathological examination of bone marrow biopsy revealed scattered interstitial infiltration of cerebriform lymphocytes（Sézary cells）,CD2（weakly positive,W＋）,CD3（W＋）,CD4（W＋）,CD8（-）,CD5（W＋）,CD7（loss of immunophenotype）,CD43（W＋）,and the proliferative index of Ki-67 was 35%. Pathological diagnosis of Sézary＇s syndrome with bone marrow involvement was made.
出处
《中国皮肤性病学杂志》
CSCD
北大核心
2017年第12期1337-1341,共5页
The Chinese Journal of Dermatovenereology
