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Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature
Erdheim–Chester disease (ECD) is a rare, multiorgan, non-Langerhans cell histiocytosis of uncertain origin, characterized by systemic xanthogranulomatous infiltration from CD68+CD1a- histiocytes. Skeletal involvement is present in up to 96% of cases with bilateral osteosclerosis of meta-diaphysis of...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4985366/ https://www.ncbi.nlm.nih.gov/pubmed/26512552 http://dx.doi.org/10.1097/MD.0000000000001365 |
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author | Nicolazzi, Maria Anna Carnicelli, Annamaria Fuorlo, Mariella Favuzzi, Angela Maria Rita Landolfi, Raffaele |
author_facet | Nicolazzi, Maria Anna Carnicelli, Annamaria Fuorlo, Mariella Favuzzi, Angela Maria Rita Landolfi, Raffaele |
author_sort | Nicolazzi, Maria Anna |
collection | PubMed |
description | Erdheim–Chester disease (ECD) is a rare, multiorgan, non-Langerhans cell histiocytosis of uncertain origin, characterized by systemic xanthogranulomatous infiltration from CD68+CD1a- histiocytes. Skeletal involvement is present in up to 96% of cases with bilateral osteosclerosis of meta-diaphysis of long bones. Furthermore, in more than 50% of cases there is 1 extraskeletal manifestation. In this case report, we describe an interesting case of ECD with an extensive pan-cardiac and vascular involvement, in addition to skeletal, retro-orbital, and retroperitoneum one. A 44-year-old woman with a long history of exophthalmos referred to our hospital for elective surgical orbital decompression. At preoperative examinations a large pericardial effusion was discovered. Echocardiography, computed tomography (CT), and magnetic resonance imaging (MRI) described an inhomogeneous mass involving pericardium and the right heart, abdominal aorta and its main branches and the retroperitoneum, suggestive for a systemic inflammatory disorder. Histological examination on a biopsy sample confirmed the diagnosis of ECD. Radiology showed the pathognomonic long-bone involvement. Surgical orbital decompression was performed and medical therapy with interferon-α (INF-α) was started. Among extraskeletal manifestations of ECD, cardiovascular involvement is often asymptomatic and thus under-diagnosed but linked to poor prognosis. This is why clinician should always look for it when a new case of ECD is diagnosed. |
format | Online Article Text |
id | pubmed-4985366 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-49853662016-08-26 Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature Nicolazzi, Maria Anna Carnicelli, Annamaria Fuorlo, Mariella Favuzzi, Angela Maria Rita Landolfi, Raffaele Medicine (Baltimore) 3400 Erdheim–Chester disease (ECD) is a rare, multiorgan, non-Langerhans cell histiocytosis of uncertain origin, characterized by systemic xanthogranulomatous infiltration from CD68+CD1a- histiocytes. Skeletal involvement is present in up to 96% of cases with bilateral osteosclerosis of meta-diaphysis of long bones. Furthermore, in more than 50% of cases there is 1 extraskeletal manifestation. In this case report, we describe an interesting case of ECD with an extensive pan-cardiac and vascular involvement, in addition to skeletal, retro-orbital, and retroperitoneum one. A 44-year-old woman with a long history of exophthalmos referred to our hospital for elective surgical orbital decompression. At preoperative examinations a large pericardial effusion was discovered. Echocardiography, computed tomography (CT), and magnetic resonance imaging (MRI) described an inhomogeneous mass involving pericardium and the right heart, abdominal aorta and its main branches and the retroperitoneum, suggestive for a systemic inflammatory disorder. Histological examination on a biopsy sample confirmed the diagnosis of ECD. Radiology showed the pathognomonic long-bone involvement. Surgical orbital decompression was performed and medical therapy with interferon-α (INF-α) was started. Among extraskeletal manifestations of ECD, cardiovascular involvement is often asymptomatic and thus under-diagnosed but linked to poor prognosis. This is why clinician should always look for it when a new case of ECD is diagnosed. Wolters Kluwer Health 2015-10-30 /pmc/articles/PMC4985366/ /pubmed/26512552 http://dx.doi.org/10.1097/MD.0000000000001365 Text en Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 |
spellingShingle | 3400 Nicolazzi, Maria Anna Carnicelli, Annamaria Fuorlo, Mariella Favuzzi, Angela Maria Rita Landolfi, Raffaele Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title | Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title_full | Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title_fullStr | Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title_full_unstemmed | Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title_short | Cardiovascular Involvement in Erdheim–Chester Disease: A Case Report and Review of the Literature |
title_sort | cardiovascular involvement in erdheim–chester disease: a case report and review of the literature |
topic | 3400 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4985366/ https://www.ncbi.nlm.nih.gov/pubmed/26512552 http://dx.doi.org/10.1097/MD.0000000000001365 |
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