Adam Z, Stepánková S, Sirotková A, Cermáková Z, Pour L, Krejcí M, Zahradová L, Korístek Z, Lenz J, Hájek R, Vorlícek J, Mayer J
Interní hematoonkologická klinika Lékarské fakulty MU a FN Brno.
Vnitr Lek. 2011 Feb;57(2):214-21.
We describe a case of an untreated female patient monitored over 8 years for chronic B-lymphocytic leukaemia (B-CLL). Over the 8 years, the patient has gradually developed severe kidney failure, even though the criteria for B-CLL treatment had not been fulfilled. Kidney biopsy revealed renal damage due to lamda free light chains cast nephropathy as well as an infiltration of renal parenchyma with B-CLL cells. It was not before this biopsy that the presence of monoclonal immunoglobulins has been investigated. Immunofixation identified free monoclonal lamda light chains in the serum and urine. Their serum concentration, quantified by densitometry, was 2.6 g/l and urine concentration was 0.5 g/l. A specific evaluation of free light chains in the serum revealed an extremely high concentration of free X light chains, over 4500 mg/l, and normal concentration of K free light chains, 10 mg/l. The aim of this report is to emphasise that monoclonal immunoglobulin may be present in B-CLL as well as other lymphoprolipherative diseases and that it may cause damage to organs, similar to multiple myeloma or monoclonal gammopathy of undetermined significance. The described case confirms poor prognostic value of monoclonal immunoglobulin free light chains in patients with B-CLL and usefulness of an evaluation of their presence in patients with B-CLL, particularly if the patients have increased creatinine level. The described case also highlights the need for evaluation of the presence of free light chains in the serum of all patients with unclear cause of renal failure.
我们描述了一例未经治疗的女性慢性B淋巴细胞白血病(B-CLL)患者,对其进行了8年的监测。在这8年中,尽管尚未达到B-CLL的治疗标准,但患者逐渐发展为严重肾衰竭。肾活检显示,肾损害是由λ游离轻链管型肾病以及B-CLL细胞浸润肾实质所致。直到此次活检才对单克隆免疫球蛋白的存在进行了调查。免疫固定电泳在血清和尿液中鉴定出游离单克隆λ轻链。通过光密度法测定,其血清浓度为2.6g/l,尿液浓度为0.5g/l。血清中游离轻链的特异性评估显示,游离λ轻链浓度极高,超过4500mg/l,而游离κ轻链浓度正常,为10mg/l。本报告的目的是强调单克隆免疫球蛋白可能存在于B-CLL以及其他淋巴增殖性疾病中,并且它可能像多发性骨髓瘤或意义未明的单克隆丙种球蛋白病一样对器官造成损害。所描述的病例证实了单克隆免疫球蛋白游离轻链在B-CLL患者中的预后价值较差,以及评估其在B-CLL患者中存在情况的实用性,特别是当患者肌酐水平升高时。所描述的病例还强调了对所有肾衰竭病因不明患者的血清中游离轻链存在情况进行评估的必要性。