BPDCN白血病患者骨髓涂片肿瘤细胞形态学特点分析

Morphological characteristics of bone marrow smears from patients with BPDCN leukemia

目的分析母细胞性浆细胞样树突细胞肿瘤(BPDCN)白血病患者骨髓涂片肿瘤细胞形态学特点。方法采用回顾性研究,收集100例BPDCN白血病患者的临床资料,对患者骨髓涂片标本进行收集。于油镜下观察BPDCN白血病患者肿瘤细胞胞体、胞核、胞浆及染色质等形态学特点。结果100例患者均出现皮肤受累,脾大77例(77.00%),肝大68例(68.00%),淋巴结肿大81例(81.00%)。发病时均存在外周血象异常,多为贫血,23例患者伴有全血细胞减少;62例患者外周血涂片出现类似异常幼稚细胞。典型BPDCN细胞存在明显异质性,核不规则形或圆形,中等大小,胞浆灰蓝色,浆内无颗粒,染色质丝网状,呈现不均匀弱嗜碱性,沿胞膜下出现珍珠项链样排列的空泡,特征性伪足突出;BPDCN细胞可大致分为急性淋巴瘤细胞白血病样、急性淋巴细胞白血病样和急性单核细胞白血病样三种类似形态。BPDCN白血病患者过氧化物酶均为阴性,糖原染色均为阳性,且呈现大块状、颗粒状或圆珠样阳性。免疫表型分析结果显示,骨髓中肿瘤细胞均表达CD123、CD38、CD56、CD4及HLA-DR,部分患者表达CD13、CD33,不表达c MPO、CD3、CD15、CD34、CD11b、CD14、CD8、CD20、CD19、CD10、CD22。结论BPDCN细胞存在特征性的形态学表现,细胞形态可大致分为淋巴瘤细胞样、原幼淋巴细胞样和原始单核细胞样;如若患者存在皮肤受累,则存在BPDCN白血病的可能,临床中可通过病理活检和流式细胞学检查以进一步明确诊断。

Objective To analyze the morphological characteristics of bone marrow smears from patients with blastocytic plasmacytoid dendritic cell neoplasm(BPDCN)leukemia.Methods The clinical data of 100 patients with BPDCN leukemia in our hospital were collected retrospectively.Bone marrow smear specimens were collected.Morphological characteristics of tumor cell bodies,nuclei,cytoplasm and chromatin in BPDCN leukemia patients were observed under oil microscopy.Results Skin involvement occurred in all 100 patients(100%),splenomegaly in 77 cases(77.00%),hepatomegaly in 68 cases(68.00%)and lymphadenopathy in 81 cases(81.00%).There were abnormal peripheral blood picture in all patients,most of them were anemia.There were 23 cases of pancytopenia and 62 cases of abnormal peripheral blood smears with juvenile cells.Typical BPDCN cells have obvious heterogeneity,irregular or round nuclei,medium size,gray-blue cytoplasm,no granules in the plasma,fibrous chromatin,uneven weak basophilic,and Pearl necklace-like vacuoles along the cell membrane with prominent characteristic pseudopodia.BPDCN cells can be roughly divided into acute lymphoma cell leukemia-like,acute lymphocyte leukemia-like and acute monocyte-like.There are three similar forms of nuclear cell leukemia.In BPDCN leukemia patients,peroxidase was negative,glycogen staining was positive,and showed large,granular or ball-like positive.Immunophenotypic analysis showed that CD123,CD38,CD56,CD4 and HLA-DR were expressed in bone marrow tumors,while CD13 and CD33 were expressed in some patients,but not cMPO,CD3,CD15,CD34,CD11b,CD14,CD8,CD20,CD19,CD10 and CD22.Conclusion There are characteristic morphological manifestations of BPDCN cells,which can be roughly divided into lymphoma cell-like,projuvenile lymphocyte-like and primitive monocyte-like.If patients have skin involvement,there is the possibility of BPDCN leukemia,which can be further diagnosed clinically by pathological biopsy and flow cytology.