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目的探讨母细胞性浆细胞样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm,BPDCN)的临床表现、免疫表型以及治疗。方法回顾性分析1例BPDCN患者临床资料并文献复习。结果患者为69岁的男性,以皮肤多发结节伴淋巴结肿大为首发表现。肿瘤细胞表达CD4、CD56、CDl23。PET-CT提示淋巴结、骨髓、脾脏均有侵犯。初始予CHOP方案化疗敏感,短期内复发后对CHOP方案耐药,改用GDP、MA、CAG方案化疗,效果均欠佳。结论 BPDCN好发于老年男性,具有独特的免疫表型。BPDCN是一种高度恶性的造血系统肿瘤,除侵及皮肤外,还侵犯骨髓、淋巴结等,预后极差。
Objective To investigate the clinical manifestations, immunophenotype and treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Methods The clinical data and literature review of 1 BPDCN were retrospectively analyzed. Results The patient was 69 years old and showed multiple nodules with enlarged lymph nodes. Tumor cells express CD4, CD56, and CDl23. PET-CT tips lymph nodes, bone marrow, spleen are violations. Initial CHOP regimen chemotherapy sensitive, short-term recurrence of CHOP program resistant to switch to GDP, MA, CAG regimen chemotherapy, the effect is poor. Conclusion BPDCN is more common in older men and has unique immunophenotype. BPDCN is a highly malignant hematopoietic tumor, in addition to invasion of the skin, but also violations of bone marrow, lymph nodes, poor prognosis.