Spectrum of monoclonal light-chain gammopathy in a tertiary care hospital

M. Zafar, S. Sinha, S. Aggarwal, M. Bhargava
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Abstract

Background: Monoclonal light chain gammopathies are uncommon subsets of plasma cell disorders which usually present as diagnostic challenge. Materials and Methods: Twenty cases of monoclonal light-chain gammopathy were identified after screening 150 plasma cell disorders at a tertiary care referral center of North India and were analyzed for clinical profile and treatment outcomes. Results: Out of 20 cases of monoclonal light-chain gammopathy, 65% (13/20) were light-chain multiple myeloma (LCMM) type, 20% (4/20) were light-chain deposition disease (LCDD) type, and 15% (3/20) had primary amyloidosis (AL). Renal failure (65% of cases) was the most common presentation. All the patients with LCDD presented with renal failure (4/4) while as 61% of LCMM (8/13) and 33% of AL (1/3) presented with renal failure. Five patients presented with anemia and all were LCMM type. Two patients presented with lytic bone lesions (LCMM type) and one patient presented with plasmacytoma (LCMM). Overall response rate after 4 cycles of induction therapy was 92.3% in LCMM group, 100% in LCDD group, and 33.33% in AL group (excluding one patient who expired before the start of treatment). LCMM showed 23% partial remission (PR), 30.77% very good PR, 38.46% complete response (CR), and 5% no response (NR). LCDD showed PR 75% and CR 25%. AL showed PR 33.33% and NR 5%. Conclusions: Renal failure is a common presentation of monoclonal light chain gammopathies, and it should alert the treating physician for the underlying uncommon plasma cell disorder.
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某三级医院单克隆轻链伽玛病谱分析
背景:单克隆轻链伽玛病是罕见的浆细胞疾病亚群,通常是诊断上的挑战。材料和方法:在北印度三级保健转诊中心对150例浆细胞疾病进行筛查后,发现了20例单克隆轻链伽玛病,并分析了临床资料和治疗结果。结果:20例单克隆轻链γ病中,65%(13/20)为轻链多发性骨髓瘤(LCMM)型,20%(4/20)为轻链沉积病(LCDD)型,15%(3/20)为原发性淀粉样变性(AL)。肾衰竭(65%的病例)是最常见的表现。LCDD患者均出现肾功能衰竭(4/4),LCMM患者有61% (8/13),AL患者有33%(1/3)出现肾功能衰竭。5例患者出现贫血,均为LCMM型。2例患者出现溶解性骨病变(LCMM型),1例患者出现浆细胞瘤(LCMM)。诱导治疗4个周期后,LCMM组总有效率为92.3%,LCDD组为100%,AL组为33.33%(不包括1例开始治疗前死亡的患者)。LCMM患者部分缓解率为23%,非常好缓解率为30.77%,完全缓解率为38.46%,无缓解率为5%。lcd显示PR 75%, CR 25%。AL显示PR为33.33%,NR为5%。结论:肾功能衰竭是单克隆轻链伽玛病的常见表现,应提醒治疗医师注意潜在的罕见浆细胞疾病。
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审稿时长
31 weeks
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