Refractory ascites as a presenting feature of extramedullary plasmacytoma in an end-stage renal disease patient with HIV infection.

Clinical Nephrology. Case Studies Pub Date : 2019-02-10 eCollection Date: 2019-01-01 DOI:10.5414/CNCS109560
Karim Soliman, Johann Herberth, Tibor Fülöp, Angie Duong, Rachel L Sturdivant
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Abstract

Refractory ascites as the only presenting feature of an extramedullary plasmacytoma complicating end-stage renal disease and HIV infection has not been described yet. We describe a case of a 39-year-old female with HIV-associated nephropathy manifesting with ascites formation after transition from peritoneal dialysis (PD) to hemodialysis (HD). Earlier on, she received cycler-assisted PD for 5 years uneventfully. A few weeks after HD transition, a striking refractory ascites developed requiring multiple paracenteses (5 - 7 L every second week). Serum protein electrophoresis showed hypoalbuminemia with only small amount of monoclonal IgG-κ at 0.30 g/dL. Serum immunofixation electrophoresis showed polyclonal immunoglobulins with polyclonal light chains. Both κ and λ light chains were increased, at 66.86 mg/dL (reference range: 0.33 - 1.94) and 18.55 mg/dL (reference range: 0.57 - 2.63), respectively, with a ratio of 3.6 (reference range: 0.26 - 1.65). However, an ascitic fluid analysis showed a marked increase in plasma cells with a κ : λ ratio greater than 5 : 1. Omental biopsy confirmed κ-restricted plasma cells. Multiple myeloma work-up with skeletal survey showed no evidence of focal osseous lesions, while bone marrow aspiration and biopsy also remained unremarkable. Accordingly, the diagnosis of omental extramedullary plasmacytoma with malignant ascites was confirmed. Conversion from PD to HD may unmask an underlying pathology favoring ascites formation.

Abstract Image

难治性腹水是终末期肾病伴HIV感染患者髓外浆细胞瘤的一个表现特征。
难治性腹水是髓外浆细胞瘤合并终末期肾脏疾病和HIV感染的唯一表现特征,目前尚未报道。我们描述了一个39岁的女性艾滋病相关肾病从腹膜透析(PD)过渡到血液透析(HD)后表现为腹水形成。在此之前,她接受了5年的自行车辅助PD治疗。HD转变后几周,出现了严重的难治性腹水,需要多次穿刺(每两周5 - 7l)。血清蛋白电泳显示低白蛋白血症,仅有少量单克隆IgG-κ在0.30 g/dL。血清免疫固定电泳显示多克隆免疫球蛋白具有多克隆轻链。κ和λ轻链均增加,分别为66.86 mg/dL(参考范围:0.33 ~ 1.94)和18.55 mg/dL(参考范围:0.57 ~ 2.63),比值为3.6(参考范围:0.26 ~ 1.65)。然而,腹水分析显示浆细胞明显增加,κ: λ比大于5:1。网膜活检证实为κ-限制性浆细胞。多发性骨髓瘤的骨骼检查显示没有局灶性骨病变的证据,而骨髓穿刺和活检也没有明显变化。因此,诊断为大网膜髓外浆细胞瘤合并恶性腹水。从PD到HD的转变可能揭示了有利于腹水形成的潜在病理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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