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Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy
Major histocompatibility complex class II (MHC II) is essential for adaptive immune response. We recently reported on disturbed adaptive mucosal immunity due to MHC II deficiency and prolonged enteropathy. Here, we share medical history, flow cytometric analysis of blood lymphocytes, immunohistopath...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6736774/ https://www.ncbi.nlm.nih.gov/pubmed/31516959 http://dx.doi.org/10.1016/j.dib.2019.104446 |
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author | Posovszky, Carsten Sirin, Mehtap Jacobsen, Eva Lorenz, Myriam Schwarz, Klaus Schmidt-Choudhury, Anjona Schütz, Catharina Hönig, Manfred Debatin, Klaus-Michael Schulz, Ansgar Möller, Peter Barth, Thomas F. |
author_facet | Posovszky, Carsten Sirin, Mehtap Jacobsen, Eva Lorenz, Myriam Schwarz, Klaus Schmidt-Choudhury, Anjona Schütz, Catharina Hönig, Manfred Debatin, Klaus-Michael Schulz, Ansgar Möller, Peter Barth, Thomas F. |
author_sort | Posovszky, Carsten |
collection | PubMed |
description | Major histocompatibility complex class II (MHC II) is essential for adaptive immune response. We recently reported on disturbed adaptive mucosal immunity due to MHC II deficiency and prolonged enteropathy. Here, we share medical history, flow cytometric analysis of blood lymphocytes, immunohistopathology, and fecal analysis of seven genetically confirmed patients with MHC II deficiency suffering from enteropathy. Data on flow cytometric analysis of HLA-DR expression on monocytes and B cells before hematopoietic stem cell transplantation (HSCT) and after in-vitro stimulation is shown. The course of immune reconstitution after HSCT of MHC II deficient patients in comparison to severe combined immunodeficiency (SCID) patients is described. In addition, immunohistopathology illustrating CD4 and CD8 T cell infiltration, absence of B lymphocytes and plasma cells, and disturbed immunoglobulin expression in the gut as well as absent HLA-DR expression in the liver is shown. Furthermore, data from fecal analysis such as stool fat, nitrogen, and water fraction as well as faecal markers such as alpha-1-antitrypsin, pancreas specific elastase 1, eosinophilic protein X (EPX), and beta defensin 2 are presented. Altogether this data demonstrates the complex phenotype of MHC II deficiency. The data can be valuable for researchers interested in mucosal immunity. For further interpretation of the data presented in this article, please see the research article “Persisting enteropathy and disturbed adaptive mucosal immunity due to MHC class II deficiency” (Posovszky et al., 2019). |
format | Online Article Text |
id | pubmed-6736774 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-67367742019-09-12 Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy Posovszky, Carsten Sirin, Mehtap Jacobsen, Eva Lorenz, Myriam Schwarz, Klaus Schmidt-Choudhury, Anjona Schütz, Catharina Hönig, Manfred Debatin, Klaus-Michael Schulz, Ansgar Möller, Peter Barth, Thomas F. Data Brief Medicine and Dentistry Major histocompatibility complex class II (MHC II) is essential for adaptive immune response. We recently reported on disturbed adaptive mucosal immunity due to MHC II deficiency and prolonged enteropathy. Here, we share medical history, flow cytometric analysis of blood lymphocytes, immunohistopathology, and fecal analysis of seven genetically confirmed patients with MHC II deficiency suffering from enteropathy. Data on flow cytometric analysis of HLA-DR expression on monocytes and B cells before hematopoietic stem cell transplantation (HSCT) and after in-vitro stimulation is shown. The course of immune reconstitution after HSCT of MHC II deficient patients in comparison to severe combined immunodeficiency (SCID) patients is described. In addition, immunohistopathology illustrating CD4 and CD8 T cell infiltration, absence of B lymphocytes and plasma cells, and disturbed immunoglobulin expression in the gut as well as absent HLA-DR expression in the liver is shown. Furthermore, data from fecal analysis such as stool fat, nitrogen, and water fraction as well as faecal markers such as alpha-1-antitrypsin, pancreas specific elastase 1, eosinophilic protein X (EPX), and beta defensin 2 are presented. Altogether this data demonstrates the complex phenotype of MHC II deficiency. The data can be valuable for researchers interested in mucosal immunity. For further interpretation of the data presented in this article, please see the research article “Persisting enteropathy and disturbed adaptive mucosal immunity due to MHC class II deficiency” (Posovszky et al., 2019). Elsevier 2019-08-28 /pmc/articles/PMC6736774/ /pubmed/31516959 http://dx.doi.org/10.1016/j.dib.2019.104446 Text en © 2019 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Medicine and Dentistry Posovszky, Carsten Sirin, Mehtap Jacobsen, Eva Lorenz, Myriam Schwarz, Klaus Schmidt-Choudhury, Anjona Schütz, Catharina Hönig, Manfred Debatin, Klaus-Michael Schulz, Ansgar Möller, Peter Barth, Thomas F. Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title | Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title_full | Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title_fullStr | Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title_full_unstemmed | Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title_short | Dataset of clinical, immunohistopathological and laboratory features of patients with MHC II deficiency suffering from enteropathy |
title_sort | dataset of clinical, immunohistopathological and laboratory features of patients with mhc ii deficiency suffering from enteropathy |
topic | Medicine and Dentistry |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6736774/ https://www.ncbi.nlm.nih.gov/pubmed/31516959 http://dx.doi.org/10.1016/j.dib.2019.104446 |
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