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首页> 外文期刊>Journal of clinical and experimental hematopathology : >Simultaneous presentation of Waldenstr?m macroglobulinemia and multiple myeloma: multidisciplinary diagnosis, treatment and 30-month follow-up.
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Simultaneous presentation of Waldenstr?m macroglobulinemia and multiple myeloma: multidisciplinary diagnosis, treatment and 30-month follow-up.

机译:Waldenstr?大球蛋白血症和多发性骨髓瘤的同时表现:多学科诊断,治疗和30个月的随访。

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摘要

Waldenstr?m macroglobulinemia and multiple myeloma are mature B-cell neoplasms deriving from post-germinal cells at different stages of differentiation. The simultaneous presentation of Waldenstr?m macroglobulinemia and multiple myeloma in the same patient is a very rare phenomenon and, so far, only two cases have been described. We report the case of a 75-year Caucasian female patient, with a silent clinical history, who presented with anemia and two different monoclonal proteins (IgMκ and IgGκ). The trephine biopsy showed the presence of a dual population, represented by small lymphoplasmacytoid cells and by plasma cells, which infiltrated the bone marrow with a clearly different pattern. Both immunohistochemistry and flow cytometry demonstrated the biclonal origin such neoplastic cells, since lymphoplasmacytoid cells resulted IgMκ while plasma cells were IgGκ. This biclonal pattern was further confirmed by the demonstration of a different IgH gene rearrangement of the two neoplasms. The patient was treated with bortezomib, dexamethasone and rituximab, achieving partial remission of both Waldenstr?m macroglobulinemia and multiple myeloma. After a 30-month follow-up, she is in stable disease. Multiple myeloma has been described in association with other indolent B-cell neoplasms, mostly chronic lymphocytic leukemia, while Waldenstr?m macroglobulinemia can be followed by diffuse large B-cell lymphoma in some instances, after chemotherapy. The association of Waldenstr?m macroglobulinemia and multiple myeloma seems to be very rare. Our study shows that an integrated diagnostic work-up is very useful in such cases, with an interesting role for flow cytometry. [J Clin Exp Hematop 53(1): 29-36, 2013].
机译:Waldenstr?巨球蛋白血症和多发性骨髓瘤是成熟的B细胞肿瘤,起源于不同分化阶段的发芽后细胞。在同一例患者中同时出现华氏巨球蛋白血症和多发性骨髓瘤是一种非常罕见的现象,到目前为止,仅描述了两例。我们报告了一位75岁的白人女性患者的病例,该患者具有无声的临床病史,患有贫血和两种不同的单克隆蛋白(IgMκ和IgGκ)。对苯丙氨酸活检显示存在双重人群,以小的淋巴浆细胞样细胞和浆细胞代表,其以明显不同的模式浸润骨髓。免疫组织化学和流式细胞术都证明了肿瘤细胞是双克隆起源的,因为淋巴浆细胞样细胞产生IgMκ而浆细胞是IgGκ。通过证明两种肿瘤具有不同的IgH基因重排,进一步证实了这种双曲线模式。该患者接受了硼替佐米,地塞米松和利妥昔单抗治疗,使沃尔登斯特罗姆巨球蛋白血症和多发性骨髓瘤均部分缓解。经过30个月的随访,她的病情稳定。已描述多发性骨髓瘤与其他惰性B细胞肿瘤(主要是慢性淋巴细胞性白血病)相关,而在化疗后,Waldenstr?m巨球蛋白血症可继发于弥漫性大B细胞淋巴瘤。 Waldenstr?m巨球蛋白血症与多发性骨髓瘤的关联似乎非常罕见。我们的研究表明,在此类情况下,综合诊断检查非常有用,并且对流式细胞仪具有重要作用。 [J Clin Exp Hematop 53(1):29-36,2013]。

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