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Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature

Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-specific clinical and l...

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Autores principales: Fattizzo, Bruno, Ferraresi, Marta, Giannotta, Juri Alessandro, Barcellini, Wilma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7923749/
https://www.ncbi.nlm.nih.gov/pubmed/33672504
http://dx.doi.org/10.3390/jcm10040870
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author Fattizzo, Bruno
Ferraresi, Marta
Giannotta, Juri Alessandro
Barcellini, Wilma
author_facet Fattizzo, Bruno
Ferraresi, Marta
Giannotta, Juri Alessandro
Barcellini, Wilma
author_sort Fattizzo, Bruno
collection PubMed
description Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-specific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lymphoproliferative disorders. In the present article, we describe two patients presenting at the emergency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma. We discuss the diagnostic challenges in the differential diagnosis of acute cytopenias in the internal medicine setting, providing a literature review of secondary HLH and AIC.
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spelling pubmed-79237492021-03-03 Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature Fattizzo, Bruno Ferraresi, Marta Giannotta, Juri Alessandro Barcellini, Wilma J Clin Med Article Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-specific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lymphoproliferative disorders. In the present article, we describe two patients presenting at the emergency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma. We discuss the diagnostic challenges in the differential diagnosis of acute cytopenias in the internal medicine setting, providing a literature review of secondary HLH and AIC. MDPI 2021-02-20 /pmc/articles/PMC7923749/ /pubmed/33672504 http://dx.doi.org/10.3390/jcm10040870 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Fattizzo, Bruno
Ferraresi, Marta
Giannotta, Juri Alessandro
Barcellini, Wilma
Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title_full Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title_fullStr Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title_full_unstemmed Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title_short Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
title_sort secondary hemophagocytic lymphohistiocytosis and autoimmune cytopenias: case description and review of the literature
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7923749/
https://www.ncbi.nlm.nih.gov/pubmed/33672504
http://dx.doi.org/10.3390/jcm10040870
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