母细胞性浆细胞样树突细胞肿瘤临床病理学观察

Blastic plasmacytoid dendritic cell neoplasm： a clinicopathologic study

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。

Objective To study the clinieopathologic 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 3 were died 5 and 2 months after diagnosis, respectively. Conclusion Blastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologie and immunophenotypie features.