title: Monoclonal gammopathy of renal significance with light-chain deposition disease diagnosed postrenal transplant: a diagnostic and therapeutic challenge.
abstract: 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.
authors.affiliation: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Aruna
authors.initials: A
authors.lastname: Nambirajan
authors.forename: Dipankar
authors.initials: D
authors.lastname: Bhowmik
authors.forename: Geetika
authors.initials: G
authors.lastname: Singh
authors.forename: Sanjay Kumar
authors.initials: SK
authors.lastname: Agarwal
authors.forename: Amit Kumar
authors.initials: AK
authors.lastname: Dinda
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D000069286:Bortezomib / Q000627:therapeutic use*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000601:surgery; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy
chemical_list: D000970:Antineoplastic Agents; D007147:Immunoglobulin Light Chains; D000069286:Bortezomib
keywords: MGRS; bortezomib; light-chain deposition disease; myeloma; transplant