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Indolent systemic mastocytosis associated with light chain deposition disease

Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast cells in one or more organ systems. SM in association with plasma cell dyscrasia is very rare. We report a first case of indolent SM (ISM) associated with light chain deposition disease (LCDD) in a kidney biopsy from a 59...

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Autores principales: Sasaki, Kotaro, Chang, Alice, Najafian, Behzad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4432416/
https://www.ncbi.nlm.nih.gov/pubmed/26019820
http://dx.doi.org/10.1093/ckj/sfs104
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author Sasaki, Kotaro
Chang, Alice
Najafian, Behzad
author_facet Sasaki, Kotaro
Chang, Alice
Najafian, Behzad
author_sort Sasaki, Kotaro
collection PubMed
description Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast cells in one or more organ systems. SM in association with plasma cell dyscrasia is very rare. We report a first case of indolent SM (ISM) associated with light chain deposition disease (LCDD) in a kidney biopsy from a 59-year-old female presenting with skin rash, elevated serum creatinine, hematuria and mild proteinuria. Subsequent workup demonstrated IgG kappa monoclonal protein in serum and urine. A bone marrow biopsy revealed neoplastic mast cells involving bone marrow without evidence of clonal myeloid or lymphoid proliferation. Kidney biopsy demonstrated modest mesangial expansion detected by light microscopy and unequivocal evidence of monoclonal kappa light chain deposition within glomerular capillaries, tubular basement membranes and vascular walls detected by immunofluorescence and/or electron microscopy, along with equivocal evidence of light chain cast nephropathy. Despite treatment with bortezomib and dexamethasone, her renal function was progressively declined over the next 6 months. This case is a reminder that SM can coincide with LCDD, which requires clinical suspicion and multimodality workup on a kidney biopsy including immunofluorescence and electron microscopy to reach the correct diagnosis.
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spelling pubmed-44324162015-05-27 Indolent systemic mastocytosis associated with light chain deposition disease Sasaki, Kotaro Chang, Alice Najafian, Behzad Clin Kidney J Original Contributions Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast cells in one or more organ systems. SM in association with plasma cell dyscrasia is very rare. We report a first case of indolent SM (ISM) associated with light chain deposition disease (LCDD) in a kidney biopsy from a 59-year-old female presenting with skin rash, elevated serum creatinine, hematuria and mild proteinuria. Subsequent workup demonstrated IgG kappa monoclonal protein in serum and urine. A bone marrow biopsy revealed neoplastic mast cells involving bone marrow without evidence of clonal myeloid or lymphoid proliferation. Kidney biopsy demonstrated modest mesangial expansion detected by light microscopy and unequivocal evidence of monoclonal kappa light chain deposition within glomerular capillaries, tubular basement membranes and vascular walls detected by immunofluorescence and/or electron microscopy, along with equivocal evidence of light chain cast nephropathy. Despite treatment with bortezomib and dexamethasone, her renal function was progressively declined over the next 6 months. This case is a reminder that SM can coincide with LCDD, which requires clinical suspicion and multimodality workup on a kidney biopsy including immunofluorescence and electron microscopy to reach the correct diagnosis. Oxford University Press 2012-10 /pmc/articles/PMC4432416/ /pubmed/26019820 http://dx.doi.org/10.1093/ckj/sfs104 Text en © The Author 2012. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please email: journals.permissions@oup.com http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Original Contributions
Sasaki, Kotaro
Chang, Alice
Najafian, Behzad
Indolent systemic mastocytosis associated with light chain deposition disease
title Indolent systemic mastocytosis associated with light chain deposition disease
title_full Indolent systemic mastocytosis associated with light chain deposition disease
title_fullStr Indolent systemic mastocytosis associated with light chain deposition disease
title_full_unstemmed Indolent systemic mastocytosis associated with light chain deposition disease
title_short Indolent systemic mastocytosis associated with light chain deposition disease
title_sort indolent systemic mastocytosis associated with light chain deposition disease
topic Original Contributions
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4432416/
https://www.ncbi.nlm.nih.gov/pubmed/26019820
http://dx.doi.org/10.1093/ckj/sfs104
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