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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...

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Autores principales: Gnanasambandam, Janani, Geminiganesan, Sangeetha, Vilvanathan, Vairan, Sankaranarayanan, Shuba, Padmanabhan, Ramachandran
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Communications and Publications Division (CPD) of the IFCC 2022
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.
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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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