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A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma

Patient: Male, 31 Final Diagnosis: Light chain depsotion disease Symptoms: — Medication: — Clinical Procedure: None Specialty: Hematology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin...

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Autores principales: Mohan, Meera, Gokden, Murat, Gokden, Neriman, Schinke, Carolina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4801155/
https://www.ncbi.nlm.nih.gov/pubmed/26988342
http://dx.doi.org/10.12659/AJCR.895762
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author Mohan, Meera
Gokden, Murat
Gokden, Neriman
Schinke, Carolina
author_facet Mohan, Meera
Gokden, Murat
Gokden, Neriman
Schinke, Carolina
author_sort Mohan, Meera
collection PubMed
description Patient: Male, 31 Final Diagnosis: Light chain depsotion disease Symptoms: — Medication: — Clinical Procedure: None Specialty: Hematology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease (CIDD), is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known. CASE REPORT: We present the case of a 31-year-old man with a solitary thoracic plasmacytoma and cardiac light chain deposition disease with evidence of congestive heart failure by echocardiography and cardiac markers. The patient underwent surgical resection of the plasmacytoma followed by systemic therapy with 50% VDT-PACE and then VRD with near-normalization of his heart function. A melphalan-based stem cell transplant is planned in this young patient to achieve the best possible long-term remission. CONCLUSIONS: CIDD is a very rare disease, with previous reports showing diverse manifestations with variable outcome. A high level of clinical suspicion should be maintained in such cases and early intervention, as in our patient, can restore cardiac function. There is very little literature on the optimal management of these patients. A combination of surgery and chemotherapy were pursued in our patient with very good results.
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spelling pubmed-48011552016-04-05 A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma Mohan, Meera Gokden, Murat Gokden, Neriman Schinke, Carolina Am J Case Rep Articles Patient: Male, 31 Final Diagnosis: Light chain depsotion disease Symptoms: — Medication: — Clinical Procedure: None Specialty: Hematology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease (CIDD), is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known. CASE REPORT: We present the case of a 31-year-old man with a solitary thoracic plasmacytoma and cardiac light chain deposition disease with evidence of congestive heart failure by echocardiography and cardiac markers. The patient underwent surgical resection of the plasmacytoma followed by systemic therapy with 50% VDT-PACE and then VRD with near-normalization of his heart function. A melphalan-based stem cell transplant is planned in this young patient to achieve the best possible long-term remission. CONCLUSIONS: CIDD is a very rare disease, with previous reports showing diverse manifestations with variable outcome. A high level of clinical suspicion should be maintained in such cases and early intervention, as in our patient, can restore cardiac function. There is very little literature on the optimal management of these patients. A combination of surgery and chemotherapy were pursued in our patient with very good results. International Scientific Literature, Inc. 2016-03-18 /pmc/articles/PMC4801155/ /pubmed/26988342 http://dx.doi.org/10.12659/AJCR.895762 Text en © Am J Case Rep, 2016 This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License
spellingShingle Articles
Mohan, Meera
Gokden, Murat
Gokden, Neriman
Schinke, Carolina
A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title_full A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title_fullStr A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title_full_unstemmed A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title_short A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma
title_sort case of cardiac light chain deposition disease in a patient with solitary plasmacytoma
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4801155/
https://www.ncbi.nlm.nih.gov/pubmed/26988342
http://dx.doi.org/10.12659/AJCR.895762
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