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EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease

BACKGROUND: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this...

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Autores principales: Kinross-Wright, John Jacob, Potu, Kalyan Chakravarthy, Pownell, Brandy, Lamfers, Randall, Bleeker, Jonathan S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5859865/
https://www.ncbi.nlm.nih.gov/pubmed/29692937
http://dx.doi.org/10.1155/2018/6063519
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author Kinross-Wright, John Jacob
Potu, Kalyan Chakravarthy
Pownell, Brandy
Lamfers, Randall
Bleeker, Jonathan S.
author_facet Kinross-Wright, John Jacob
Potu, Kalyan Chakravarthy
Pownell, Brandy
Lamfers, Randall
Bleeker, Jonathan S.
author_sort Kinross-Wright, John Jacob
collection PubMed
description BACKGROUND: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. CASE SUMMARY: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation. She underwent evaluation for occult malignancy and was found to have diffuse intra-abdominal mesenteric lymphadenopathy on CT scan. Biopsy of mesenteric nodes revealed an EBV positive, CD20 positive mixed lymphoproliferative process with T-cell predominance, but without a monoclonal cell population felt to be consistent with EBV-associated LPD. Bone marrow biopsy revealed hemophagocytic lymphohistiocytosis, complicating her course. She was treated with steroids and rituximab but continued to decline, eventually developing MSSA bacteremia and succumbing to her disease. CONCLUSION: To our knowledge, this is the first report of the constellation of celiac sprue, EBV-associated LPD, and hemophagocytic lymphohistiocytosis. Providers caring for patients with severe, uncontrolled celiac disease and adenopathy should consider EBV-associated LPD.
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spelling pubmed-58598652018-04-24 EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease Kinross-Wright, John Jacob Potu, Kalyan Chakravarthy Pownell, Brandy Lamfers, Randall Bleeker, Jonathan S. Case Rep Hematol Case Report BACKGROUND: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. CASE SUMMARY: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation. She underwent evaluation for occult malignancy and was found to have diffuse intra-abdominal mesenteric lymphadenopathy on CT scan. Biopsy of mesenteric nodes revealed an EBV positive, CD20 positive mixed lymphoproliferative process with T-cell predominance, but without a monoclonal cell population felt to be consistent with EBV-associated LPD. Bone marrow biopsy revealed hemophagocytic lymphohistiocytosis, complicating her course. She was treated with steroids and rituximab but continued to decline, eventually developing MSSA bacteremia and succumbing to her disease. CONCLUSION: To our knowledge, this is the first report of the constellation of celiac sprue, EBV-associated LPD, and hemophagocytic lymphohistiocytosis. Providers caring for patients with severe, uncontrolled celiac disease and adenopathy should consider EBV-associated LPD. Hindawi 2018-03-05 /pmc/articles/PMC5859865/ /pubmed/29692937 http://dx.doi.org/10.1155/2018/6063519 Text en Copyright © 2018 John Jacob Kinross-Wright et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kinross-Wright, John Jacob
Potu, Kalyan Chakravarthy
Pownell, Brandy
Lamfers, Randall
Bleeker, Jonathan S.
EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title_full EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title_fullStr EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title_full_unstemmed EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title_short EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease
title_sort ebv-associated lymphoproliferative disorder and hemophagocytic lymphohistiocytosis in a patient with severe celiac disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5859865/
https://www.ncbi.nlm.nih.gov/pubmed/29692937
http://dx.doi.org/10.1155/2018/6063519
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