Waldenström’s Macroglobulinemia and Cryoglobulinemic Glomerulonephritis: An Unusual Case of Monoclonal Gammopathy of Renal Significance

J. C. De la Flor, Jesús de María Sulca, Pablo Rodríguez, Daniel Villa, Edna Sandoval, Rocío Zamora, Maribel Monroy-Condori, Roxana Lipa, Henry Perez, Michael Cieza
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Abstract

Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström’s macroglobulinemia. We present the case of a 52-year-old patient with a history of cutaneous vasculitis and hypothyroidism, who presented with generalized edema, moderate anemia, hypercholesterolemia, nephrotic range proteinuria of 12.69 g/day, microhematuria, arterial hypertension, and hypocomplementemia via the classical pathway, without acute kidney injury and with negative serological studies and positive cryoglobulins in the second determination. Serum and urine protein electrophoresis and immunofixation studies showed a monoclonal band of IgM and kappa light chain. Renal biopsy was consistent with cryoglobulinemic glomerulonephritis. In the context of dysproteinemia and cryoglobulinemic glomerulonephritis, bone-marrow aspiration and biopsy were performed, leading to the diagnosis of Waldenström’s macroglobulinemia. Monoclonal gammopathies have been described in association with type I cryoglobulinemias. This described association is uncommon, which is why we present this case, along with a review of the literature.
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瓦尔登斯特伦巨球蛋白血症和冷球蛋白血症肾小球肾炎:具有肾脏意义的单克隆丙种球蛋白病的罕见病例
低温球蛋白是免疫球蛋白,在低于37℃的温度下沉淀,再加热时溶解。它们可诱发累及肾脏的小血管炎。低温球蛋白血症性肾小球肾炎是一种罕见的表现,发生在单克隆γ病患者中,特别是Waldenström的巨球蛋白血症。我们报告一例52岁的患者,有皮肤血管炎和甲状腺功能减退病史,表现为全身性水肿,中度贫血,高胆固醇血症,肾病范围蛋白尿12.69 g/天,微量血尿,动脉高血压,经经典途径补体不足,无急性肾损伤,血清学研究阴性,第二次测定冷球蛋白阳性。血清和尿蛋白电泳和免疫固定研究显示IgM和kappa轻链单克隆带。肾活检符合冷球蛋白血症性肾小球肾炎。在蛋白异常血症和冷球蛋白血症性肾小球肾炎的情况下,进行骨髓穿刺和活检,诊断为Waldenström的巨球蛋白血症。单克隆伽玛病已被描述为与I型冷球蛋白血症相关。这种描述的关联是不常见的,这就是为什么我们提出这个案例,以及文献综述。
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