Nambirajan Aruna, Bhowmik Dipankar, Singh Geetika, Agarwal Sanjay Kumar, Dinda Amit Kumar
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Transpl Int. 2015 Mar;28(3):375-9. doi: 10.1111/tri.12497. Epub 2014 Dec 16.
Patients with light-chain deposition disease (LCDD) frequently do not meet criteria for myeloma. In such cases, despite low tumor burden, the circulating monoclonal immunoglobulins cause renal damage, are responsible for post-transplant recurrence, and are rightly categorized as monoclonal gammopathy of renal significance (MGRS) requiring chemotherapy. A 65-year male with uncharacterized nodular glomerulopathy presented with proteinuria 3 years postrenal transplant. His allograft biopsies were diagnostic of light-chain deposition disease (likely recurrent), and in the absence of myeloma, he was labeled as MGRS. Based on the limited literature available, he was treated with bortezomib which resulted in normalization of serum-free light-chain ratios and resolution of proteinuria. He, however, later succumbed to complications of chemotherapy. This case highlights the diagnostic difficulties in LCDD, the importance of an accurate pretransplant diagnosis, and treatment of the malignant clone, in the absence of which post-transplant management of recurrence is challenging with poor outcomes.
轻链沉积病(LCDD)患者常常不符合骨髓瘤的诊断标准。在此类病例中,尽管肿瘤负荷较低,但循环中的单克隆免疫球蛋白会导致肾脏损害,是移植后复发的原因,并且被正确归类为具有肾脏意义的单克隆丙种球蛋白病(MGRS),需要进行化疗。一名65岁患有未明确诊断的结节性肾小球病的男性患者在肾移植3年后出现蛋白尿。他的移植肾活检诊断为轻链沉积病(可能为复发性),且在无骨髓瘤的情况下,他被归类为MGRS。基于现有的有限文献,他接受了硼替佐米治疗,结果血清游离轻链比值恢复正常,蛋白尿消失。然而,他后来死于化疗并发症。该病例突出了LCDD的诊断困难、准确的移植前诊断的重要性以及恶性克隆的治疗,若缺乏这些,移植后复发的管理具有挑战性且预后不良。