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Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases
Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophago...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Communications and Publications Division (CPD) of the IFCC
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9092716/ https://www.ncbi.nlm.nih.gov/pubmed/35645690 |
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author | Gnanasambandam, Janani Geminiganesan, Sangeetha Vilvanathan, Vairan Sankaranarayanan, Shuba Padmanabhan, Ramachandran |
author_facet | Gnanasambandam, Janani Geminiganesan, Sangeetha Vilvanathan, Vairan Sankaranarayanan, Shuba Padmanabhan, Ramachandran |
author_sort | Gnanasambandam, Janani |
collection | PubMed |
description | Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophagocytic lymphohistiocytosis which were secondary to typhoid and dengue fever. While primary disease occurs predominantly in infants, secondary hemophagocytic lymphohistiocytosis can occur in any age group. Both primary and secondary hemophagocytic lymphohistiocytosis are characterised by fever, hepatosplenomegaly, pancytopenia and multiorgan dysfunction. But unusual persistence of fever and other organ involvement should need further workup for hemophagocytic lymphohistiocytosis. Secondary hemophagocytic lymphohistiocytosis may resolve on treating the underlying disorder. But severe cases need treatment with immunosuppressive/immunomodulation therapy to prevent morbidity. Early clinical suspicion, prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis are essential to prevent deleterious effects to health. |
format | Online Article Text |
id | pubmed-9092716 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | The Communications and Publications Division (CPD) of the IFCC |
record_format | MEDLINE/PubMed |
spelling | pubmed-90927162022-05-27 Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases Gnanasambandam, Janani Geminiganesan, Sangeetha Vilvanathan, Vairan Sankaranarayanan, Shuba Padmanabhan, Ramachandran EJIFCC Case Study Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophagocytic lymphohistiocytosis which were secondary to typhoid and dengue fever. While primary disease occurs predominantly in infants, secondary hemophagocytic lymphohistiocytosis can occur in any age group. Both primary and secondary hemophagocytic lymphohistiocytosis are characterised by fever, hepatosplenomegaly, pancytopenia and multiorgan dysfunction. But unusual persistence of fever and other organ involvement should need further workup for hemophagocytic lymphohistiocytosis. Secondary hemophagocytic lymphohistiocytosis may resolve on treating the underlying disorder. But severe cases need treatment with immunosuppressive/immunomodulation therapy to prevent morbidity. Early clinical suspicion, prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis are essential to prevent deleterious effects to health. The Communications and Publications Division (CPD) of the IFCC 2022-04-11 /pmc/articles/PMC9092716/ /pubmed/35645690 Text en Copyright © 2022 International Federation of Clinical Chemistry and Laboratory Medicine (IFCC). All rights reserved. https://creativecommons.org/licenses/by-nc/4.0/This is a Platinum Open Access Journal distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Study Gnanasambandam, Janani Geminiganesan, Sangeetha Vilvanathan, Vairan Sankaranarayanan, Shuba Padmanabhan, Ramachandran Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title | Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title_full | Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title_fullStr | Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title_full_unstemmed | Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title_short | Secondary Hemophagocytic Lymphohistiocytosis – A Common Ramification of Different Diseases |
title_sort | secondary hemophagocytic lymphohistiocytosis – a common ramification of different diseases |
topic | Case Study |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9092716/ https://www.ncbi.nlm.nih.gov/pubmed/35645690 |
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