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轻链型骨髓瘤:来自印度的一份简要报告。

Light chain myeloma: A brief report from India.

作者信息

Singh Neha, Agrawal Narendra, Sekhri Radhika, Mehta Anurag, Kumar Dushyant, Vishwakarma Gayatri, Ahmed Rayaz, Bhurani Dinesh

机构信息

Department of Pathology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India.

Department of Hemato-Oncology, Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India.

出版信息

Indian J Pathol Microbiol. 2019 Jul-Sep;62(3):441-444. doi: 10.4103/IJPM.IJPM_385_18.

Abstract

Light chain myeloma (LCM) has a reported worldwide incidence of approximately 15%-20% among all multiple myeloma (MM) patients. Few western studies have shown strong correlation of LCM with anemia, higher International Staging System scores, proclivity to renal failure, elevated lactate dehydrogenase levels, raised serum-free light chain ratio, higher frequency of extramedullary plasmacytomas, and poorer overall survival, attributable probably to lack of differentiation and skeletal destruction. The primary aim of this retrospective observational study was to define the clinical and hematological characteristics as well as prognostic outcome of Indian LCM patients in comparison with the IgG and IgA subtypes. Patients were defined according to the International Myeloma Working Group diagnostic criteria 2016 and staged as per the International Staging System. Out of 104 patients of newly diagnosed MM in which results of serum immunofixation (IFE) were available, 65 were of IgG isotype (62.5%), 15 had IgA (14.4%), and 24 had light chain myelomas (LCMs) (23.1%). It was observed that LCM patients significantly correlated with hypercalcemia and higher serum-free light chain ratios, whereas IgA patients were strongly associated with anemia and lower serum albumin levels. However, no difference was found among the three subgroups in terms of serum lactate dehydrogenase levels, proclivity to renal failure, presence of lytic bone lesions, prognostic scoring, pretransplant chemosensitivity, and progession-free survival (1 year). Thus, it may be concluded that Indian LCM patients have significantly different clinico-hematological profile in comparison with other published studies worldwide. Also, their prognostic outcomes are not worse when compared with patients of other protein isotypes, probably due to standardized treatment regimens applied.

摘要

据报道,在所有多发性骨髓瘤(MM)患者中,轻链骨髓瘤(LCM)在全球的发病率约为15%-20%。很少有西方研究表明LCM与贫血、较高的国际分期系统评分、肾衰竭倾向、乳酸脱氢酶水平升高、血清游离轻链比值升高、髓外浆细胞瘤发生率较高以及总体生存率较差密切相关,这可能归因于缺乏分化和骨骼破坏。这项回顾性观察研究的主要目的是确定印度LCM患者与IgG和IgA亚型相比的临床和血液学特征以及预后结果。患者根据国际骨髓瘤工作组2016年诊断标准进行定义,并按照国际分期系统进行分期。在104例可获得血清免疫固定电泳(IFE)结果的新诊断MM患者中,65例为IgG亚型(62.5%),15例为IgA亚型(14.4%),24例为轻链骨髓瘤(LCM)(23.1%)。观察到LCM患者与高钙血症和较高的血清游离轻链比值显著相关,而IgA患者与贫血和较低的血清白蛋白水平密切相关。然而,在血清乳酸脱氢酶水平、肾衰竭倾向、溶骨性骨病变的存在、预后评分、移植前化疗敏感性和无进展生存期(1年)方面,三个亚组之间没有差异。因此,可以得出结论,与全球其他已发表的研究相比,印度LCM患者具有明显不同的临床血液学特征。此外,与其他蛋白亚型的患者相比,他们的预后结果并不更差,这可能是由于采用了标准化的治疗方案。

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