Kidney involvement in Idiopathic Light Chain disease. Report of one case

• Published in: Revista medíca de Chile 2013-03, Vol.141 (3), p.396-401
• Main Authors: Trimarchi, Hernán, Lombi, Fernando, Forrester, Mariano, Pomeranz, Vanesa, Rabinovich, Oscar, Stemmelin, Germán R, Ruiz, Pablo A, Iotti, Alejandro, Young, Pablo
• Format: Article
• Language: Spanish
• Subjects: Diabetic Nephropathies - etiology; Diabetic Nephropathies - pathology; Humans; Immunoglobulin Light Chains - analysis; Male; Middle Aged; Paraproteinemias - complications; Paraproteinemias - pathology
• ISSN: 0717-6163
• DOI: 10.4067/S0034-98872013000300018

Idiopathic Light Chain disease (ILCD) is a systemic disease characterized by a deposit in different organs of light chain monoclonal immunoglobulins, produced by an abnormal clone of B cells. It is usually found in the course ofa plasma cell dyscrasia and in other lymphoproliferative alterations; however it may occur in absence of any hematologic disease and is denominated as idiopathic. We report a 51-year-old mole admitted to the hospital due to anasarca. Laboratory evaluation showed a serum creatinine of 1.4 mg/dl, a serum albumin of1.6 g/dl, a serum cholesterol of 687 mg/dl and a proteinuria of 5.3 g/day Light chains with a predominance of a monoclonal component were identified in urinary proteins by electrophoresis and kappa chains were identified by immunofixation. A renal biopsy showed a diffuse nodular glomerulopathy with a 35% tubular atrophy and interstitial sclerosis. Electrón microscopy confirmed light chain deposition. The bone marrow biopsy showed a myeloid hyperplasia. The patient was initially treated with methylprednisolone and plasmapheresis with a reduction in serum creatinine and disappearance of urinary kappa component. Albuminuria persisted and a malnutrition-inflammatory complex syndrome was diagnosed. Hemodialysis with ultrafiltration was started along with cyclophosphamide. The patient received hemodialysis for six months and continued with methylprednisolone.