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...
Main Authors: | , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Hindawi Limited
2018-01-01
|
Series: | Case Reports in Hematology |
Online Access: | http://dx.doi.org/10.1155/2018/6063519 |
id |
doaj-e7f436b2b02340a2a8e912c747887591 |
---|---|
record_format |
Article |
spelling |
doaj-e7f436b2b02340a2a8e912c7478875912020-11-24T20:44:14ZengHindawi LimitedCase Reports in Hematology2090-65602090-65792018-01-01201810.1155/2018/60635196063519EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac DiseaseJohn Jacob Kinross-Wright0Kalyan Chakravarthy Potu1Brandy Pownell2Randall Lamfers3Jonathan S. Bleeker4Department of Internal Medicine, Sanford USD School of Medicine, Sioux Falls, SD, USADepartment of Internal Medicine, Sanford USD School of Medicine, Sioux Falls, SD, USADepartment of Pathology, Sanford USD School of Medicine, Sioux Falls, SD, USADepartment of Internal Medicine, Sanford USD School of Medicine, Sioux Falls, SD, USADivision of Hematology and Oncology, Sanford USD School of Medicine, Sioux Falls, SD, USABackground. 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.http://dx.doi.org/10.1155/2018/6063519 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
John Jacob Kinross-Wright Kalyan Chakravarthy Potu Brandy Pownell Randall Lamfers Jonathan S. Bleeker |
spellingShingle |
John Jacob Kinross-Wright Kalyan Chakravarthy Potu Brandy Pownell Randall Lamfers Jonathan S. Bleeker EBV-Associated Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis in a Patient with Severe Celiac Disease Case Reports in Hematology |
author_facet |
John Jacob Kinross-Wright Kalyan Chakravarthy Potu Brandy Pownell Randall Lamfers Jonathan S. Bleeker |
author_sort |
John Jacob Kinross-Wright |
title |
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_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_sort |
ebv-associated lymphoproliferative disorder and hemophagocytic lymphohistiocytosis in a patient with severe celiac disease |
publisher |
Hindawi Limited |
series |
Case Reports in Hematology |
issn |
2090-6560 2090-6579 |
publishDate |
2018-01-01 |
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. |
url |
http://dx.doi.org/10.1155/2018/6063519 |
work_keys_str_mv |
AT johnjacobkinrosswright ebvassociatedlymphoproliferativedisorderandhemophagocyticlymphohistiocytosisinapatientwithsevereceliacdisease AT kalyanchakravarthypotu ebvassociatedlymphoproliferativedisorderandhemophagocyticlymphohistiocytosisinapatientwithsevereceliacdisease AT brandypownell ebvassociatedlymphoproliferativedisorderandhemophagocyticlymphohistiocytosisinapatientwithsevereceliacdisease AT randalllamfers ebvassociatedlymphoproliferativedisorderandhemophagocyticlymphohistiocytosisinapatientwithsevereceliacdisease AT jonathansbleeker ebvassociatedlymphoproliferativedisorderandhemophagocyticlymphohistiocytosisinapatientwithsevereceliacdisease |
_version_ |
1716818068141768704 |