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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-02-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/4/870 |
| _version_ | 1797395882266591232 |
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| author | Bruno Fattizzo Marta Ferraresi Juri Alessandro Giannotta Wilma Barcellini |
| author_facet | Bruno Fattizzo Marta Ferraresi Juri Alessandro Giannotta Wilma Barcellini |
| author_sort | Bruno Fattizzo |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T00:42:14Z |
| format | Article |
| id | doaj.art-f673f5d075d543418b19777d53d6d224 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T00:42:14Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-f673f5d075d543418b19777d53d6d2242023-12-11T17:46:09ZengMDPI AGJournal of Clinical Medicine2077-03832021-02-0110487010.3390/jcm10040870Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the LiteratureBruno Fattizzo0Marta Ferraresi1Juri Alessandro Giannotta2Wilma Barcellini3Oncohematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyDepartment of Internal Medicine, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyOncohematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyOncohematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyHemophagocytic 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.https://www.mdpi.com/2077-0383/10/4/870autoimmune hemolytic anemiaimmune thrombocytopeniahemophagocytic lymphohistiocytosisdiffuse large B cell lymphoma |
| spellingShingle | Bruno Fattizzo Marta Ferraresi Juri Alessandro Giannotta Wilma Barcellini Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature Journal of Clinical Medicine autoimmune hemolytic anemia immune thrombocytopenia hemophagocytic lymphohistiocytosis diffuse large B cell lymphoma |
| 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 | autoimmune hemolytic anemia immune thrombocytopenia hemophagocytic lymphohistiocytosis diffuse large B cell lymphoma |
| url | https://www.mdpi.com/2077-0383/10/4/870 |
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