Cargando…
A rare case of renal thrombotic microangiopathy associated with Castleman’s disease
BACKGROUND: Castleman’s disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report...
Autores principales: | , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2017
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5301347/ https://www.ncbi.nlm.nih.gov/pubmed/28183278 http://dx.doi.org/10.1186/s12882-017-0472-2 |
_version_ | 1782506345941958656 |
---|---|
author | Mutneja, Anubha Cossey, L. Nicholas Liapis, Helen Chen, Ying Maggie |
author_facet | Mutneja, Anubha Cossey, L. Nicholas Liapis, Helen Chen, Ying Maggie |
author_sort | Mutneja, Anubha |
collection | PubMed |
description | BACKGROUND: Castleman’s disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report a rare case of renal thrombotic microangiopathy (TMA) associated with CD and investigate the podocyte expression of VEGF in the renal biopsy prior to initiation of treatment. CASE PRESENTATION: An 18-year-old male presented with fever, diarrhea, diffuse lymphadenopathy, ascites and acute kidney injury. Laboratory tests for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura were negative. The kidney biopsy showed TMA. An excisional lymph node biopsy was consistent with CD, plasma cell variant. Immunofluorescence staining showed suppressed podocyte VEGF expression. Chemotherapy that inhibits production of inflammatory mediators including IL-6 and VEGF led to complete recovery of renal function. CONCLUSIONS: Our case illustrates a rare renal histological feature of CD. IL-6 and VEGF are postulated to suppress glomerular VEGF expression, thereby causing renal TMA. Therapy directed against these inflammatory mediators may have important therapeutic implications. |
format | Online Article Text |
id | pubmed-5301347 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-53013472017-02-15 A rare case of renal thrombotic microangiopathy associated with Castleman’s disease Mutneja, Anubha Cossey, L. Nicholas Liapis, Helen Chen, Ying Maggie BMC Nephrol Case Report BACKGROUND: Castleman’s disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report a rare case of renal thrombotic microangiopathy (TMA) associated with CD and investigate the podocyte expression of VEGF in the renal biopsy prior to initiation of treatment. CASE PRESENTATION: An 18-year-old male presented with fever, diarrhea, diffuse lymphadenopathy, ascites and acute kidney injury. Laboratory tests for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura were negative. The kidney biopsy showed TMA. An excisional lymph node biopsy was consistent with CD, plasma cell variant. Immunofluorescence staining showed suppressed podocyte VEGF expression. Chemotherapy that inhibits production of inflammatory mediators including IL-6 and VEGF led to complete recovery of renal function. CONCLUSIONS: Our case illustrates a rare renal histological feature of CD. IL-6 and VEGF are postulated to suppress glomerular VEGF expression, thereby causing renal TMA. Therapy directed against these inflammatory mediators may have important therapeutic implications. BioMed Central 2017-02-10 /pmc/articles/PMC5301347/ /pubmed/28183278 http://dx.doi.org/10.1186/s12882-017-0472-2 Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Mutneja, Anubha Cossey, L. Nicholas Liapis, Helen Chen, Ying Maggie A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title | A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title_full | A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title_fullStr | A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title_full_unstemmed | A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title_short | A rare case of renal thrombotic microangiopathy associated with Castleman’s disease |
title_sort | rare case of renal thrombotic microangiopathy associated with castleman’s disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5301347/ https://www.ncbi.nlm.nih.gov/pubmed/28183278 http://dx.doi.org/10.1186/s12882-017-0472-2 |
work_keys_str_mv | AT mutnejaanubha ararecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT cosseylnicholas ararecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT liapishelen ararecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT chenyingmaggie ararecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT mutnejaanubha rarecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT cosseylnicholas rarecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT liapishelen rarecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease AT chenyingmaggie rarecaseofrenalthromboticmicroangiopathyassociatedwithcastlemansdisease |