母细胞性浆细胞样树突细胞肿瘤2例并文献复习

Blastic plasmacytoid dendritic cell neoplasm:Two cases and literature review

目的探讨母细胞性浆细胞样树突细胞肿瘤的临床病理特征、治疗及预后。方法结合相关文献,回顾性分析2例母细胞性浆细胞样树突细胞肿瘤患者的临床表现、组织学形态、免疫表型、治疗方案及预后。结果2例组织形态均符合母细胞性浆细胞样树突细胞肿瘤的病理改变,免疫组织标记CD4、CD56、CD123、TCL1阳性,排除淋巴系、髓系及NK细胞来源,2例EBER原位杂交检测结果均为阴性;例l累及骨髓及淋巴结,对症支持治疗,2月后死亡;例2经CHOP方案化疗8周期,半年后病情进展,全身多发红疹,后予吉西他滨、奥沙利铂及培门冬酶化疗9周期,目前病情稳定。结论母细胞性浆细胞样树突细胞肿瘤多以皮肤症状为首发,具有独特的临床病理特点,诊断依靠组织学及免疫表型,目前尚无有效的治疗方案且预后差。

Objective To explore the clinical and pathological characteristics,treatment,and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Methods Based on literature review,we retrospectively analyzed clinical manifestations,histological morphology,immune phenotype,treatment plan,and prognosis of two patients with BPDCN.Results The tissue morphology of both cases was consistent with the pathological changes in BPDCN.Immunohistochemical markers cluster of differentiation(CD)4,CD56,CD123,T-cell leukemia-1(TCL1)were positively stained,and lymphoid,myeloid,and NK cell sources were ruled out.Epstein-Barr virus-encoded RNA(EBER)in situ hybridization results of both cases were negative.Case 1 involved bone marrow and lymph nodes,received symptomatic supportive treatment,and died 2 months later.Case 2 was managed by 8 cycles of chemotherapy with the cyclophosphamide,doxorubicin,vincristine,and prednisolone(CHOP)regimen.The condition of Case 2 progressed at 6 months with multiple rubella throughout the body.Later,gemcitabine,oxaliplatin,and pemetrexed chemotherapy were administered for 9 cycles.Currently,the condition of Case 2 was stable.Conclusion BPDCN often have skin symptoms as the first symptom and unique clinical and pathological characteristics.Diagnosis of BPDCN relies on histology and immunophenotype.There is currently no effective treatment plan,leading to a poor prognosis of BPDCN.