Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico

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 ofB cells. It is usually found in the course ofa plasma cell dyscrasia and in other lymphoproliferative alterations; how...

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Autores principales: Trimarchi,Hernán, Lombi,Fernando, Forrester,Mariano, Pomeranz,Vanesa, Rabinovich,Oscar, Stemmelin,Germán R, Ruiz,Pablo A, Iotti,Alejandro, Young,Pablo
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2013
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000300018
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spelling oai:scielo:S0034-988720130003000182013-11-15Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínicoTrimarchi,HernánLombi,FernandoForrester,MarianoPomeranz,VanesaRabinovich,OscarStemmelin,Germán RRuiz,Pablo AIotti,AlejandroYoung,Pablo Immunoglobulin Light chains Proteinuria Plasmapheresis Renal dialysis 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 ofB 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. Thepatient was initially treated with methylprednisolone and plasmapheresis with a reduction in serum creatinine and disappearance of urinary kappa component. Albuminuriapersisted and a malnutrition-inflammatory complex syndrome was diagnosed. Hemodialysis with ultrafiltration was started along with cyclophosphamide. Thepatient receivedhemodialysisforsixmonths and continued with methylprednisolone.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.141 n.3 20132013-03-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000300018es10.4067/S0034-98872013000300018
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Immunoglobulin Light chains
Proteinuria
Plasmapheresis
Renal dialysis
spellingShingle Immunoglobulin Light chains
Proteinuria
Plasmapheresis
Renal dialysis
Trimarchi,Hernán
Lombi,Fernando
Forrester,Mariano
Pomeranz,Vanesa
Rabinovich,Oscar
Stemmelin,Germán R
Ruiz,Pablo A
Iotti,Alejandro
Young,Pablo
Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
description 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 ofB 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. Thepatient was initially treated with methylprednisolone and plasmapheresis with a reduction in serum creatinine and disappearance of urinary kappa component. Albuminuriapersisted and a malnutrition-inflammatory complex syndrome was diagnosed. Hemodialysis with ultrafiltration was started along with cyclophosphamide. Thepatient receivedhemodialysisforsixmonths and continued with methylprednisolone.
author Trimarchi,Hernán
Lombi,Fernando
Forrester,Mariano
Pomeranz,Vanesa
Rabinovich,Oscar
Stemmelin,Germán R
Ruiz,Pablo A
Iotti,Alejandro
Young,Pablo
author_facet Trimarchi,Hernán
Lombi,Fernando
Forrester,Mariano
Pomeranz,Vanesa
Rabinovich,Oscar
Stemmelin,Germán R
Ruiz,Pablo A
Iotti,Alejandro
Young,Pablo
author_sort Trimarchi,Hernán
title Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
title_short Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
title_full Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
title_fullStr Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
title_full_unstemmed Enfermedad renal por depósito idiopático de cadenas livianas: Caso clínico
title_sort enfermedad renal por depósito idiopático de cadenas livianas: caso clínico
publisher Sociedad Médica de Santiago
publishDate 2013
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872013000300018
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