病例报告:急性肾衰竭患者一例IgD lambda/lambda多发性骨髓瘤,血清电泳无单克隆尖峰

T. Troiano, V. Brescia, Lucia De Marinis, A. Marinaccio, R. Lovero, R. Rizzi, F. Di Serio
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摘要

IgD多发性骨髓瘤是一种罕见的浆细胞病变,约占所有多发性骨髓瘤病例的1-2%。它主要影响年轻男性受试者;与其他同型MM相比,其特点是病程积极,Bence Jones蛋白产量高,急性肾功能衰竭,结果往往令人遗憾。其显著特征是血清蛋白电泳(SPE)缺乏单克隆峰。病例报告一名57岁男性,因左下肢疼痛和体重减轻而前往急诊科(急诊科)。实验室检查显示:正红细胞正色性贫血(血红蛋白9.4 g/dL),急性肾功能衰竭(s-肌酐2.85 mg/dL, e-GFR 23 mL/min/1.73 m²)。血清蛋白电泳(SPE)仅在γ区检测到轻度多克隆,未发现单克隆峰。结果血清免疫固定(s-IFE)显示单克隆IgD λ带和单克隆λ带。自由轻链(s-FLC)的比值为0.04。骨髓活检证实克隆浆细胞浸润> 20%;肾活检诊断“铸型肾病”。结论IgD λ/λ多发性骨髓瘤是一种罕见的疾病,预后较差;早期和正确的实验室诊断对于适当的治疗和有效的监测至关重要,以改善患者的预后。
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Case Report: A case of IgD lambda/lambda Multiple Myeloma in patient with acute renal failure and without monoclonal spike in serum electrophoresis
Background IgD Multiple Myeloma is a rare form of plasma cell dyscrasia and accounts for approximately 1-2% of all cases of Multiple Myeloma. It mainly affects young, male subjects; it is characterized by an aggressive course, a high production of Bence Jones protein, acute renal failure and an often unfortunate outcome compared to the other isotypes of MM. A distinctive feature is the lack of a monoclonal peak on serum protein electrophoresis (SPE). Case report a 57-year-old man with pain in his left lower limb and weight loss goes to the Emergency Department (Emergency Department). Laboratory tests performed showed normocytic normochromic anemia (Hemoglobin 9.4 g/dL), acute renal failure (s-creatinine 2.85 mg/dL, e-GFR 23 mL/min/1.73 m². serum protein electrophoresis (SPE) detected only mild polyclonal in the gamma zone with no evidence of any monoclonal peak. Results serum immunofixation (s-IFE) showed a monoclonal IgD λ band and a monoclonal λ band. The Free Light Chains (s-FLC) measurement showed a ratio of 0.04. The bone marrow biopsy confirmed an infiltration of> 20% of clonal plasma cells; renal biopsy diagnosed “cast nephropathy”. Conclusion IgD λ/λ Multiple Myeloma is a rare form of this disease with a poor prognosis; an early and correct laboratory diagnosis is crucial for appropriate treatment and effective monitoring in order to improve patient outcome.
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