Cytogenetic Findings in a Case of Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN).

Carlos A Tirado, John Reinartz, Katherine Lapp, Diane Zhao, Andrew M Nguyen, Kevin Stieglbauer
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Abstract

Objectives: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), previously known as natural killer (NK) cell leukemia/lymphoma, is categorized by the World Health Organization as a sole entity. Most often, BPDCN presents with features of both lymphoma and leukemia. The average age at diagnosis is 60 to 70 years and there are more men than women who are diagnosed with BPDCN. Herein we report a 67-year-old female with a recent peripheral blood morphology revealing a hematopoietic leukemia process. Flow cytometry revealed an atypical cell population without B-cell or T-cell lineage expression. It was positive for CD45 and CD123 and negative for CD34. The peripheral blood showed blastic plasmacytoid dendritic cell neoplasm, macrocytic anemia and moderate thrombocytopenia. Chromosome analysis showed an abnormal clone with i(7)(q10) and monosomies of chromosomes 13 and 15. She underwent a bone marrow biopsy. Bone marrow and peripheral blood showed a blastic plasmacytoid dendritic cell neoplasm (BPDCN), hypercellular marrow (estimated 95%) with 90.4% blasts (aspirate smear).

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1例母浆细胞样树突状细胞瘤的细胞遗传学研究。
目的:母浆细胞样树突状细胞肿瘤(BPDCN),以前被称为自然杀伤(NK)细胞白血病/淋巴瘤,被世界卫生组织列为唯一的实体。大多数情况下,BPDCN表现为淋巴瘤和白血病的特征。诊断时的平均年龄为60至70岁,被诊断为BPDCN的男性多于女性。在此,我们报告一位67岁的女性,最近外周血形态学显示造血白血病的过程。流式细胞术显示非典型细胞群无b细胞或t细胞谱系表达。CD45和CD123呈阳性,CD34呈阴性。外周血表现为母浆细胞样树突状细胞瘤、巨细胞性贫血和中度血小板减少症。染色体分析显示异常克隆为i(7)(q10),染色体13和15单体。她接受了骨髓活检。骨髓和外周血显示母细胞浆细胞样树突状细胞瘤(BPDCN),骨髓高细胞瘤(估计95%)伴90.4%的母细胞瘤(抽吸涂片)。
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