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

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
John Jacob Kinross-Wright ◽  
Kalyan Chakravarthy Potu ◽  
Brandy Pownell ◽  
Randall Lamfers ◽  
Jonathan S. Bleeker
Keyword(s):  
Celiac Disease ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Rare Condition ◽  
Lymphoproliferative Disease ◽  
Celiac Sprue ◽  
Caucasian Woman ◽  
First Report ◽  
Barr Virus ◽  
Occult Malignancy ◽  
Epstein Barr

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.

scholarly journals Hemophagocytic lymphohistiocytosis in a patient with glioblastoma: a case report

CNS Oncology ◽  
2019 ◽  
Vol 8 (4) ◽  
pp. CNS45 ◽  
Author(s):  
Vaibhav Kumar ◽  
Patrick J Eulitt ◽  
Ana Bermudez ◽  
Simon Khagi
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Hematologic Malignancy ◽  
Multiorgan Failure ◽  
Rare Condition ◽  
Solid Organ ◽  
Adult Onset ◽  
Barr Virus ◽  
Epstein Barr ◽  
High Index Of Suspicion

Adult onset hemophagocytic lymphohistiocytosis (HLH) is a rare condition, usually secondary to either a precipitating infective or hematologic malignancy. We present a case of Epstein–Barr virus associated HLH in a 55-year-old female receiving treatment for a glioblastoma (GBM). It is possible that HLH is under recognized, as patients with GBM often have features of a nonspecific systemic inflammatory response syndrome, multiorgan failure and cognitive decline. A high index of suspicion and increased awareness can help improve timeliness of diagnosis. Therapeutically, Epstein–Barr virus associated HLH in patients with solid organ malignancy poses significant challenges. An individualized, multidisciplinary approach is essential when managing adult-onset HLH and providers will need to be mindful of the high mortality rate despite treatment.

scholarly journals Epstein-Barr virus induced hemophagocytic lymphohistiocytosis in X-linked lymphoproliferative disease

2014 ◽  
Vol 5 (02) ◽  
pp. 171-174 ◽  
Author(s):  
Senthilkumar Sankararaman ◽  
Rosario Maria Riel-Romero ◽  
Majed Jeroudi ◽  
Eduardo Gonzalez-Toledo
Keyword(s):  
Magnetic Resonance ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Genetic Disorder ◽  
Lymphoproliferative Disease ◽  
Specific Pattern ◽  
Resonance Spectroscopy ◽  
Barr Virus ◽  
Epstein Barr ◽  
The Brain

ABSTRACTX-linked lymphoproliferative disease (XLP) is a rare, often fatal genetic disorder characterized by extreme vulnerability to Epstein-Barr virus (EBV). EBV-induced hemophagocytic lymphohistiocytosis (HLH) is a known presentation in XLP. In EBV-induced HLH in XLP, the brain imaging findings in the acute phase include a non specific pattern. In this report, we highlight the magnetic resonance imaging and magnetic resonance spectroscopy findings in a child with EBV induced HLH in XLP.

scholarly journals Novel ZAP-70-Related Immunodeficiency Presenting with Epstein–Barr Virus Lymphoproliferative Disorder and Hemophagocytic Lymphohistiocytosis

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Moriah Forster ◽  
Timothy Moran ◽  
Anne Beaven ◽  
Timothy Voorhees
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Severe Combined Immunodeficiency ◽  
Lymphoproliferative Disease ◽  
Receptor Complex ◽  
Combined Immunodeficiency ◽  
Barr Virus ◽  
Zeta Chain ◽  
Integral Role ◽  
Epstein Barr

Zeta-chain-associated protein kinase 70 (ZAP-70) plays an integral role in the T-cell antigenic receptor complex. A deficiency of this kinase leads to a phenotype of severe combined immunodeficiency, while hypomorphic mutations of the kinase lead to more mild immunodeficiency phenotypes. We present a case of a 21-year-old patient with lymphadenopathy who was found to have Epstein–Barr virus (EBV) lymphoproliferative disease (LPD) and the development of hemophagocytic lymphohistiocytosis (HLH). On further workup, the patient was ultimately found to have a homozygous intrionic mutation in ZAP-70. This is a novel ZAP-70 mutation (c.1623 + 5G > A) associated with combined immunodeficiency and an EBV-positive LPD. A primary immunodeficiency is important to consider in a young, otherwise healthy patient presenting with an EBV-positive LPD.

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