IgD多发性骨髓瘤:血清游离轻链试验的临床、生物学特征和预后价值

R. Djidjik , Y. Lounici , K. Chergeulaïne , Y. Berkouk , S. Mouhoub , S. Chaib , M. Belhani , M. Ghaffor
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引用次数: 13

摘要

IgD多发性骨髓瘤(MM)是一种罕见的骨髓瘤亚型,影响不到2%的MM患者。为了评估血清游离轻链(sFLCs)分析的临床和预后特征,我们检查了17例IgD MM。从1998年到2012年,我们获得了1250例单克隆γ病变,包括590例多发性骨髓瘤和17例IgD MM。男性患者占优势,平均诊断年龄为:59±12岁。IgD MM患者的生存期较短(中位生存期= 9个月)。主要表现为:骨痛(75%)、淋巴结病(16%)、肝肿大(25%)、脾肿大(8%)、相关AL淀粉样变(6%)、肾功能损害(82%)、感染(47%)、高钙血症(37%)和贫血(93%)。所有低丙种球蛋白血症患者的血清电泳显示一个微妙的m峰(平均= 13.22±10 g/L)。Lambda轻链的比例过高(65%);91%的患者血清β2-微球蛋白增高,71%的患者有本·琼斯蛋白尿。sFLCs λ κ的中位率分别为19.05 mg/L和296.75 mg/L。93%的患者sFLCR异常,基线sFLCR与生存率一致(P = 0.034)。FLC检测对IgD MM患者的预后至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
IgD multiple myeloma: Clinical, biological features and prognostic value of the serum free light chain assay

IgD multiple myeloma (MM) is a rare subtype of myeloma, it affects less than 2% of patients with MM. To evaluate the clinical and prognostic attributes of serum free light chains (sFLCs) analysis, we examined 17 cases of IgD MM. From 1998 to 2012, we obtained 1250 monoclonal gammapathies including 590 multiple myeloma and 17 patients had IgD MM. With preponderance of men patients with a mean age at diagnosis of: 59 ± 12 years. Patients with IgD MM have a short survival (Median survival = 9 months). The presenting features included: bone pain (75%), lymphadenopathy (16%), hepatomegaly (25%), splenomegaly (8%), associated AL amyloidosis (6%), renal impairment function (82%), infections (47%), hypercalcemia (37%) and anemia (93%). Serum electrophoresis showed a subtle M-spike (Mean = 13.22 ± 10 g/L) in all patients associated to a hypogammaglobulinemia. There was an over-representation of Lambda light chain (65%); high serum β2-microglobulin in 91% and Bence Jones proteinuria was identified in 71%. The median rate of sFLCs κ was 19.05 mg/L and 296.75 mg/L for sFLCs λ. sFLCR was abnormal in 93% of patients and it showed concordance between baseline sFLCR and the survival (P = 0.034). The contribution of FLC assay is crucial for the prognosis of patients with IgD MM.

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来源期刊
Pathologie-biologie
Pathologie-biologie 医学-病理学
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