CAJ | 학술논문

目的探讨母细胞性浆细胞样树突细胞肿瘤的临床特征和鉴别诊断.方法结合相关文献,回顾性分析3例母细胞性浆细胞样树突细胞肿瘤的临床表现、组织学形态和免疫表型.结果 3例组织形态符合母细胞性浆细胞样树突细胞肿瘤的病理改变,同时具有新特点,免疫组织化学可排除B系、T系、髓系和NK细胞来源,分别为CD56+ CD4- CD123+ TdT+ CD43+ CD68+、CD56+ CD4+CD123- TdT+ CD43+ CD68-和CD56+ CD4+ CD123-/+TdT-CD43+ CD68+.例1患者患病5年后累及骨髓,经化疗病情较稳定；例2和例3患者分别于发病5个月和2个月后死亡.结论母细胞性浆细胞样树突细胞肿瘤是一组异源性肿瘤,具有不同的临床特点、组织学形态和免疫表型.
목적 탐토모세포성장세포양수돌세포종류적림상특정화감별진단.방법 결합상관문헌,회고성분석3례모세포성장세포양수돌세포종류적림상표현、조직학형태화면역표형.결과 3례조직형태부합모세포성장세포양수돌세포종류적병리개변,동시구유신특점,면역조직화학가배제B계、T계、수계화NK세포래원,분별위CD56+ CD4- CD123+ TdT+ CD43+ CD68+、CD56+ CD4+CD123- TdT+ CD43+ CD68-화CD56+ CD4+ CD123-/+TdT-CD43+ CD68+.례1환자환병5년후루급골수,경화료병정교은정；례2화례3환자분별우발병5개월화2개월후사망.결론 모세포성장세포양수돌세포종류시일조이원성종류,구유불동적림상특점、조직학형태화면역표형.
Objective To study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.Methods The clinical,morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm,with review of literature.Results The pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm,and they also had new characteristics,including lineage other than T,B,myeloid and NK cells,and immunophenotypes of CD56+ CD4- CD123+ TdT+ CD43+ CD68+,CD56+ CD4 + CD123- TdT+ CD43 +CD68- and CD56+ CD4+ CD123-/+ TdT- CD43+ CD68+ in the 3 cases,respectively. Bone marrow involvement was found 5 years later in case 1,and was then stable after chemotherapy; case 2 and case 3were died 5 and 2 months after diagnosis,respectively.Conclusion Blastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders,with different clinical,morphologic and immunophenotypic features.