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非分泌型骨髓瘤:准备好迎接新定义了吗?

Non-Secretory Myeloma: Ready for a new Definition?

作者信息

Corso Alessandro, Mangiacavalli Silvia

机构信息

Clinica Ematologica, Fondazione IRCCS Policlinico San Matteo, Pavia.

出版信息

Mediterr J Hematol Infect Dis. 2017 Aug 18;9(1):e2017053. doi: 10.4084/MJHID.2017.053. eCollection 2017.

Abstract

Non-secretory myeloma is a rare myeloma subtype whose diagnosis, until a few years ago, was established by demonstration of monoclonal plasma cells ≥10% in the bone marrow and by negative results on serum and urine electrophoresis and immunofixation studies. However, this type of myeloma could be misdiagnosed if the workup does not include an accurate study of serum free light chain test since some of the patients diagnosed as non-secretory could be light chain only with small amounts monoclonal proteinuria. Due to this limit in classification, all the information available today, generally coming from retrospective studies including patients studied completely and incompletely, could be misleading. A new definition is, thus, needed to distinguish between the true non-secretory, with a possible better prognosis, and the other forms of oligo-secretory myeloma with a prognosis more similar to the secretory form of myeloma. With all the data of the literature, the availability of laboratory and radiological tools, times are mature to depict a new definition of nonsecretory myeloma that deserves a peculiar work up and different response evaluation and, may be, a different therapeutic approach.

摘要

非分泌型骨髓瘤是一种罕见的骨髓瘤亚型,直到几年前,其诊断是通过在骨髓中证实单克隆浆细胞≥10%以及血清和尿液电泳及免疫固定研究结果为阴性来确立的。然而,如果检查不包括血清游离轻链检测的准确研究,这种类型的骨髓瘤可能会被误诊,因为一些被诊断为非分泌型的患者可能仅为轻链型,伴有少量单克隆蛋白尿。由于这种分类上的局限性,目前所有可用信息(通常来自回顾性研究,包括对患者的完整和不完整研究)可能会产生误导。因此,需要一个新的定义来区分真正的非分泌型(可能预后较好)和其他形式的寡分泌型骨髓瘤(其预后更类似于分泌型骨髓瘤)。结合文献中的所有数据、实验室和放射学工具的可用性,现在是时候描绘出非分泌型骨髓瘤的新定义了,它需要特殊的检查、不同的反应评估,也许还需要不同的治疗方法。

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