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IgM单克隆丙种球蛋白病/华氏巨球蛋白血症:骨髓的形态学和免疫表型研究

IgM monoclonal gammopathy/Waldenström's macroglobulinemia: a morphological and immunophenotypic study of the bone marrow.

作者信息

Feiner H D, Rizk C C, Finfer M D, Bannan M, Gottesman S R, Chuba J V, Amorosi E

机构信息

Department of Pathology, New York University Medical Center.

出版信息

Mod Pathol. 1990 May;3(3):348-56.

PMID:2114024
Abstract

The presence of a monoclonal paraprotein in the serum or urine raises the possibility of myeloma or lymphoma/leukemia. Yet, in a significant proportion of individuals with serum paraproteins, particularly those with low levels of paraprotein, clinical and routine bone marrow evaluation is not diagnostic of an underlying neoplasm. The purpose of this study was to define the pathologic basis for macroglobulinemia in patients whose routine bone marrow biopsies were not diagnostic of a lymphoplasmacytic neoplasm. We used immunofluorescence microscopy and flow cytometry of cell suspensions prepared from aspirated marrow, as well as immunohistochemistry of core biopsies, to perform immunopathologic evaluations of the bone marrow from 16 such patients. Seven individuals without a monoclonal serum paraprotein, who were similarly studied, served as controls. Our data indicate that 13 of the 16 patients with monoclonal serum IgM paraproteins whose routine bone marrow biopsies were normal or showed nondiagnostic changes morphologically had a dispersed monotypic B lineage population of concordant immunoglobulin heavy and light chain type in the bone marrow. The immunophenotype of these cells spanned the range from mature B cell to plasmacytoid B cell to plasma cell. In four of these 13 patients a diagnosis of lymphoplasmacytic lymphoma could be made on the basis of greater than or equal to 20% monoclonal B lineage cells among bone marrow mononuclear cells.

摘要

血清或尿液中存在单克隆副蛋白会增加骨髓瘤或淋巴瘤/白血病的可能性。然而,在相当一部分血清中存在副蛋白的个体中,尤其是那些副蛋白水平较低的个体,临床和常规骨髓评估并不能诊断出潜在的肿瘤。本研究的目的是确定常规骨髓活检不能诊断为淋巴浆细胞性肿瘤的患者巨球蛋白血症的病理基础。我们使用从抽吸骨髓制备的细胞悬液进行免疫荧光显微镜检查和流式细胞术,以及对核心活检组织进行免疫组织化学检查,对16例此类患者的骨髓进行免疫病理评估。另外7例无单克隆血清副蛋白的个体也进行了类似研究,作为对照。我们的数据表明,16例血清中单克隆IgM副蛋白且常规骨髓活检正常或形态学上显示非诊断性改变的患者中,有13例骨髓中存在散在的单型B淋巴细胞群,其免疫球蛋白重链和轻链类型一致。这些细胞的免疫表型范围从成熟B细胞到浆细胞样B细胞再到浆细胞。在这13例患者中的4例中,基于骨髓单个核细胞中≥20%的单克隆B淋巴细胞群可诊断为淋巴浆细胞性淋巴瘤。

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IgM monoclonal gammopathy/Waldenström's macroglobulinemia: a morphological and immunophenotypic study of the bone marrow.IgM单克隆丙种球蛋白病/华氏巨球蛋白血症:骨髓的形态学和免疫表型研究
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Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia.华氏巨球蛋白血症的临床病理定义:第二届华氏巨球蛋白血症国际研讨会共识小组建议
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Despite apparent morphologic and immunophenotypic heterogeneity, Waldenstrom's macroglobulinemia is consistently composed of cells along a morphologic continuum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells.尽管存在明显的形态学和免疫表型异质性,但华氏巨球蛋白血症始终由沿着小淋巴细胞、浆细胞样淋巴细胞和浆细胞形态连续谱的细胞组成。
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引用本文的文献

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Waldenstrom's Macroglobulinemia: An Update.华氏巨球蛋白血症:最新进展
Mediterr J Hematol Infect Dis. 2018 Jan 1;10(1):e2018004. doi: 10.4084/MJHID.2018.004. eCollection 2018.
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Waldenström macroglobulinemia.华氏巨球蛋白血症
Curr Treat Options Oncol. 2004 Jun;5(3):239-47. doi: 10.1007/s11864-004-0015-5.