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[谱系不明确的造血细胞肿瘤的病理诊断与鉴别诊断]

[The pathological diagnosis and differential diagnosis of hematopoietic cell tumors of ambiguous lineage].

作者信息

Li M, Zhu M X, Tian L, Guo L G, Hu K, Gao Z F

机构信息

Department of Pathology, Third Hospital, School of Basic Medical Sciences, Peking University, Beijing 100191, China.

Department of Hematology, Third Hospital, Peking University, Beijing 100191, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2021 Mar 14;42(3):238-242. doi: 10.3760/cma.j.issn.0253-2727.2021.03.010.

Abstract

To explore the key points of the pathological and differential diagnoses of extra-medullary masses of hematopoietic cell tumors of ambiguous lineage, and to discuss the possible solutions. Five hematopoietic cell tumors of ambiguous lineage cases were collected, including myeloid sarcoma, mixed phenotype acute leukemia, B/myeloid, T-lymphoblastic lymphoma combined with acute myeloid leukemia, acute undifferentiated leukemia with cutaneous MPDCP and early T-precursor cell acute lymphoblastic leukemia. The data including morphology, immunostaining, and flow cytometry analysis were collected, and we explored the problems and differential diagnosis in the diagnosis of hematopoietic cell tumors of ambiguous lineage. The five cases showed that the accurate pathological diagnosis and classification of hematopoietic cell tumors of ambiguous lineage should be based on lineage-specific antigens. Moreover, tumor cells have the potential of multi-directional differentiation. In different sites or different periods, the differentiation of tumor cells may be different. Biopsy and detection of all related markers should be performed for the initial diagnosis, and the detection should be repeated when the condition of the patient changes. Combined application of multi-techniques, including morphology and flow cytometry analysis, is recommend for the diagnosis of hematopoietic cell tumors of ambiguous lineage, since the conventional morphology and immunophenotyping methods are limited. Hematopoietic cell tumors of ambiguous lineage are derived from hematopoietic stem cells with a potential of multi-differentiation. The differentiation of tumor cells is variable. We need to integrate cell morphology, flow cytometry, pathology, clinical data, and molecular genetics to make a comprehensive diagnosis.

摘要

探讨谱系不明确的造血细胞肿瘤髓外肿块的病理及鉴别诊断要点,并讨论可能的解决方法。收集5例谱系不明确的造血细胞肿瘤病例,包括髓系肉瘤、混合表型急性白血病、B/髓系、T淋巴母细胞淋巴瘤合并急性髓系白血病、伴有皮肤MPDCP的急性未分化白血病及早期T前体细胞急性淋巴细胞白血病。收集包括形态学、免疫染色及流式细胞术分析的数据,探讨谱系不明确的造血细胞肿瘤诊断中的问题及鉴别诊断。这5例病例显示,谱系不明确的造血细胞肿瘤的准确病理诊断及分类应基于谱系特异性抗原。此外,肿瘤细胞具有多向分化潜能。在不同部位或不同时期,肿瘤细胞的分化可能不同。初始诊断时应进行活检及所有相关标志物的检测,患者病情变化时应重复检测。对于谱系不明确的造血细胞肿瘤的诊断,建议联合应用包括形态学和流式细胞术分析在内的多种技术,因为传统的形态学和免疫表型分析方法存在局限性。谱系不明确的造血细胞肿瘤起源于具有多分化潜能的造血干细胞。肿瘤细胞的分化是可变的。我们需要整合细胞形态学、流式细胞术、病理学、临床数据及分子遗传学来做出综合诊断。

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