骨髓受累且无皮损表现的母细胞性浆细胞样树突细胞肿瘤1例治疗体会  

Treatment of blastocytic plasmacytoid dendritic cell tumor with bone marrow involvement and no skin lesions:a case report

作　　者：王瑞平 闫艳[1] Wang Ruiping;Yan Yan(Department of Hematology,Bayannur Hospital,Inner Mongolia Autonomous Region,Bayannur 015000,China)

机构地区：[1]内蒙古自治区巴彦淖尔市医院血液内科,巴彦淖尔015000

出　　处：《中国综合临床》2024年第2期120-122,共3页Clinical Medicine of China

摘　　要：原发性骨髓母细胞性浆细胞样树突细胞肿瘤(lastic lasmacytoid endritic ell eoplasm,BPDCN)是一种罕见的有高度侵袭性的血液系统恶性肿瘤,典型BPDCN常以皮损为首发症状,常常累及淋巴结、骨髓、中枢神经系统和其他软组织等,对化学治疗和放射治疗均不敏感,预后极差。2020年8月28日巴彦淖尔市医院收治1例以腹痛、贫血、肝脾肿大为首发表现,缺乏典型皮损症状的患者,经过骨髓形态、流式细胞免疫分型及骨髓活检检查,最终诊断BPDCN。通过对呈现无皮损表现的罕见BPDCN病例的临床资料分析,为临床医师对该病的识别及治疗提供参考。Plasm Blastic Plasmacytoid Dendritic Cell Neoplasm(BPDCN)is a rare and highly invasive hematological malignancies.Typical BPDCN usually starts with skin lesions,involves lymph nodes,bone marrow,central nervous system and other soft tissues.It is not sensitive to chemotherapy and radiotherapy,and the prognosis is very poor.A patient with abdominal pain,anemia,hepatosplenomegalosis as the first manifestations,and lack of typical skin lesions was admitted in Bayannur Hospital on August 28,2020.BPDCN was finally diagnosed by bone marrow morphology,flow cytometry and bone marrow biopsy.Provide reference for clinical doctors to indentify and treat BPDCN by analyzing the clinical data of rare BPDCN cases with no skin lesions.

关 键 词：母细胞性浆细胞样树突细胞肿瘤 骨髓 地西他滨 维奈克拉 治疗 

分 类 号：R733.3[医药卫生—肿瘤]