CAJ | 학술논문

目的探讨母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的临床病理学特征、诊断与鉴别诊断.方法对1例BPDCN患者的临床病理特征、免疫表型以及治疗和预后情况进行分析,复习文献资料.结果患者为女性,发病年龄32岁,皮肤斑块或结节.活组织检查形态学显示侵及皮肤和骨髓,镜下肿瘤细胞由小至中等大小幼稚细胞组成,细胞质稀少,核形不规则,染色质细腻,核仁不明显.免疫组织化学示肿瘤细胞CD4、CD56、CD123表达阳性,CD3、CD20、CD34、TdT、MPO表达阴性.结论 BPDCN是一种罕见的高度恶性的淋巴造血系统肿瘤,除侵及皮肤外还侵犯骨髓和淋巴结.该病预后差,应注意与其他淋巴造血系统肿瘤相鉴别.
목적 탐토모세포성장세포양수돌상세포종류(BPDCN)적림상병이학특정、진단여감별진단.방법 대1례BPDCN환자적림상병리특정、면역표형이급치료화예후정황진행분석,복습문헌자료.결과 환자위녀성,발병년령32세,피부반괴혹결절.활조직검사형태학현시침급피부화골수,경하종류세포유소지중등대소유치세포조성,세포질희소,핵형불규칙,염색질세니,핵인불명현.면역조직화학시종류세포CD4、CD56、CD123표체양성,CD3、CD20、CD34、TdT、MPO표체음성.결론 BPDCN시일충한견적고도악성적림파조혈계통종류,제침급피부외환침범골수화림파결.해병예후차,응주의여기타림파조혈계통종류상감별.
Objective To investigate the feature,pathological diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN).Methods The clinical and histological findings,immunophenotype,treatment and prognosis of one case with BPDCN were evaluated with review of the relevant literature.Results The female patient with age of 32 years presented as cutaneous lesions.Pathologic findings showed skin and bone marrow were involved.Microscopically,the tumors were composed of diffuse blasts with small to medium size,scant cytoplasm,highly irregular nuclear contours,dispersed nuclear chromatin and faint indistinct nucleolus.Immunohistochemical characteristics of the tumors were the positive expression of CD4,CD56 and CD123,and negative expression of CD3,CD20,CD34,TdT,MPO.Conclusion BPDCN is a rare and highly aggressive hematopoietic neoplasm.Except for the skin,bone marrow and lymph node can also be involved.The differential diagnosis is necessary from other haematopoietic and lymphoid tumors.