Cargando…

Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease

A 70-year-old woman with celiac disease presented with weight loss and diarrhea unresponsive to gluten-free diet (GFD) and prednisone. Diagnosis of type 2 refractory celiac disease (RCD) was made by small intestinal biopsies showing severe villous blunting and intraepithelial lymphocytosis. She was...

Descripción completa

Detalles Bibliográficos
Autores principales: Lu, Lucy, Ning, Shuoyan, Kassam, Zain, Hunt, Richard, Puglia, Marco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American College of Gastroenterology 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4435307/
https://www.ncbi.nlm.nih.gov/pubmed/26157854
http://dx.doi.org/10.14309/crj.2014.30
_version_ 1782371896471322624
author Lu, Lucy
Ning, Shuoyan
Kassam, Zain
Hunt, Richard
Puglia, Marco
author_facet Lu, Lucy
Ning, Shuoyan
Kassam, Zain
Hunt, Richard
Puglia, Marco
author_sort Lu, Lucy
collection PubMed
description A 70-year-old woman with celiac disease presented with weight loss and diarrhea unresponsive to gluten-free diet (GFD) and prednisone. Diagnosis of type 2 refractory celiac disease (RCD) was made by small intestinal biopsies showing severe villous blunting and intraepithelial lymphocytosis. She was diagnosed with hemophagocytic lymphohistiocytic syndrome (HLH) after developing fever, pancytopenia, hypofibrinogenemia, elevated ferritin, and demonstration of hemophagocytosis on her bone marrow biopsy. An expert pathologist on lymphoma reviewed her biopsies and revised the final diagnosis to type 1 enteropathy-associated T-cell lymphoma (EATL) based on large T-cells infiltrating the lamina propria. We describe the first case of HLH associated with localized EATL and RCD.
format Online
Article
Text
id pubmed-4435307
institution National Center for Biotechnology Information
language English
publishDate 2014
publisher American College of Gastroenterology
record_format MEDLINE/PubMed
spelling pubmed-44353072015-07-08 Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease Lu, Lucy Ning, Shuoyan Kassam, Zain Hunt, Richard Puglia, Marco ACG Case Rep J Case Report A 70-year-old woman with celiac disease presented with weight loss and diarrhea unresponsive to gluten-free diet (GFD) and prednisone. Diagnosis of type 2 refractory celiac disease (RCD) was made by small intestinal biopsies showing severe villous blunting and intraepithelial lymphocytosis. She was diagnosed with hemophagocytic lymphohistiocytic syndrome (HLH) after developing fever, pancytopenia, hypofibrinogenemia, elevated ferritin, and demonstration of hemophagocytosis on her bone marrow biopsy. An expert pathologist on lymphoma reviewed her biopsies and revised the final diagnosis to type 1 enteropathy-associated T-cell lymphoma (EATL) based on large T-cells infiltrating the lamina propria. We describe the first case of HLH associated with localized EATL and RCD. American College of Gastroenterology 2014-04-04 /pmc/articles/PMC4435307/ /pubmed/26157854 http://dx.doi.org/10.14309/crj.2014.30 Text en Copyright © Lu et al. This is an open-access article. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/
spellingShingle Case Report
Lu, Lucy
Ning, Shuoyan
Kassam, Zain
Hunt, Richard
Puglia, Marco
Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title_full Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title_fullStr Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title_full_unstemmed Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title_short Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
title_sort hemophagocytic lymphohistiocytic syndrome and enteropathy-associated t-cell lymphoma in a patient with refractory celiac disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4435307/
https://www.ncbi.nlm.nih.gov/pubmed/26157854
http://dx.doi.org/10.14309/crj.2014.30
work_keys_str_mv AT lulucy hemophagocyticlymphohistiocyticsyndromeandenteropathyassociatedtcelllymphomainapatientwithrefractoryceliacdisease
AT ningshuoyan hemophagocyticlymphohistiocyticsyndromeandenteropathyassociatedtcelllymphomainapatientwithrefractoryceliacdisease
AT kassamzain hemophagocyticlymphohistiocyticsyndromeandenteropathyassociatedtcelllymphomainapatientwithrefractoryceliacdisease
AT huntrichard hemophagocyticlymphohistiocyticsyndromeandenteropathyassociatedtcelllymphomainapatientwithrefractoryceliacdisease
AT pugliamarco hemophagocyticlymphohistiocyticsyndromeandenteropathyassociatedtcelllymphomainapatientwithrefractoryceliacdisease