A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper inflammatory condition which occurs as either primary (genetic) or secondary (acquired) due to impaired or absent function of Natural Killer cells and cytotoxic cells. Common secondary causes include viral and bacterial infections...

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Main Authors: Indira M, Meera Nair, Prabhalaksmi K K, Andrews M A
Format: Article
Language:English
Published: PAGEPress Publications 2014-08-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
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Online Access:https://mjhid.org/index.php/mjhid/article/view/1928
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author Indira M
Meera Nair
Prabhalaksmi K K
Andrews M A
author_facet Indira M
Meera Nair
Prabhalaksmi K K
Andrews M A
author_sort Indira M
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper inflammatory condition which occurs as either primary (genetic) or secondary (acquired) due to impaired or absent function of Natural Killer cells and cytotoxic cells. Common secondary causes include viral and bacterial infections, autoimmune diseases and hematological malignancies. Extensive phagocytosis of blood cells by histiocytes in bone marrow, spleen, liver and lymphnodes result in peripheral blood cytopenias, hepatosplenomegaly and lymphadenopathy. We evaluated a case of pyrexia of unknown origin and found out that he fulfilled the criteria for diagnosing hemophagocytic lymphohistiocytosis. He was started on immunochemotherapy with etoposide, cyclosporine and steroids, but he succumbed to illness within two weeks of treatment. High index of suspicion is needed to diagnose HLH and prompt treatment on diagnosis can be life saving
format Article
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institution OA Journals
issn 2035-3006
language English
publishDate 2014-08-01
publisher PAGEPress Publications
record_format Article
series Mediterranean Journal of Hematology and Infectious Diseases
spelling doaj-art-c80853e9ea5e4f49b4cd1b65ec404b302025-08-20T01:47:48ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062014-08-011A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown OriginIndira M0Meera Nair1Prabhalaksmi K K2Andrews M A3GMC, Thrissur, Kerala, IndiaGMC, Thrissur, Kerala, IndiaGMC, Thrissur, Kerala, IndiaGMC, Thrissur, Kerala, IndiaHemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper inflammatory condition which occurs as either primary (genetic) or secondary (acquired) due to impaired or absent function of Natural Killer cells and cytotoxic cells. Common secondary causes include viral and bacterial infections, autoimmune diseases and hematological malignancies. Extensive phagocytosis of blood cells by histiocytes in bone marrow, spleen, liver and lymphnodes result in peripheral blood cytopenias, hepatosplenomegaly and lymphadenopathy. We evaluated a case of pyrexia of unknown origin and found out that he fulfilled the criteria for diagnosing hemophagocytic lymphohistiocytosis. He was started on immunochemotherapy with etoposide, cyclosporine and steroids, but he succumbed to illness within two weeks of treatment. High index of suspicion is needed to diagnose HLH and prompt treatment on diagnosis can be life savinghttps://mjhid.org/index.php/mjhid/article/view/1928Hemophagocytic lymphohistiocytosispyrexia of unknown originimmunochemotherapy
spellingShingle Indira M
Meera Nair
Prabhalaksmi K K
Andrews M A
A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
Mediterranean Journal of Hematology and Infectious Diseases
Hemophagocytic lymphohistiocytosis
pyrexia of unknown origin
immunochemotherapy
title A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
title_full A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
title_fullStr A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
title_full_unstemmed A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
title_short A case of Hemophagocytic Lymphohistiocytosis presenting as Pyrexia of Unknown Origin
title_sort case of hemophagocytic lymphohistiocytosis presenting as pyrexia of unknown origin
topic Hemophagocytic lymphohistiocytosis
pyrexia of unknown origin
immunochemotherapy
url https://mjhid.org/index.php/mjhid/article/view/1928
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