Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication

Hemophagocytic lymphohistiocytosis (HLH) is a devastating and rare multisystemic disorder characterized by an autoimmune phenomenon leading to reactive hyperactivity of cytotoxic T cells and histiocytes mediated by cytokine storm. HLH could be primary (hereditary) or secondary (acquired). Unremitten...

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Main Authors: Ashwani K Sood, Saguna Pandit, Nidhi Chadha, Naresh I Jindal
Format: Article
Language:English
Published: Jaypee Brothers Medical Publisher 2023-12-01
Series:Pediatric Infectious Disease
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Online Access:https://www.pidjournal.com/doi/PID/pdf/10.5005/jp-journals-10081-1412
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author Ashwani K Sood
Saguna Pandit
Nidhi Chadha
Naresh I Jindal
author_facet Ashwani K Sood
Saguna Pandit
Nidhi Chadha
Naresh I Jindal
author_sort Ashwani K Sood
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a devastating and rare multisystemic disorder characterized by an autoimmune phenomenon leading to reactive hyperactivity of cytotoxic T cells and histiocytes mediated by cytokine storm. HLH could be primary (hereditary) or secondary (acquired). Unremittent fever, organomegaly, lymphadenopathy, and neurologic dysfunction are among the common manifestations of HLH, along with abnormal lab parameters like hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and transaminitis. We report a case of a 16-year-old adolescent girl presenting to the emergency department with a history of high-grade fever, with partial response to antipyretics, body aches, and severe headache for 4 days. The physical examination revealed significant hepatomegaly. enzyme-linked immunosorbent assay was positive for dengue nonstructural protein 1 (NS1) antigen (Ag) assay. The patient was managed as per the World Health Organization (WHO) protocol and started improving from dengue; however, during the second week of the illness, the patient continued to have persistent fever. A repeat lab workup revealed bicytopenia with elevated ferritin levels (17891 ng/mL). Diagnosing dengue-associated HLH is challenging unless the treating team is aware of this association, as early recognition and timely institution of immunosuppressive and specific therapy for the underlying infection is associated with improved outcomes. Physicians should collaborate with pathologists and microbiologists for early diagnosis.
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spelling doaj-art-37f34a359d6f45e2bb1aa0d56a1174ad2025-08-20T03:39:15ZengJaypee Brothers Medical PublisherPediatric Infectious Disease2582-49882023-12-015413513710.5005/jp-journals-10081-14127Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful ComplicationAshwani K Sood0Saguna Pandit1Nidhi Chadha2Naresh I Jindal3Ashwani K Sood, Department of Pediatrics, MM Institute of Medical Sciences & Research, Mullana, Haryana, India, Phone: +91 9418300888Department of Pediatrics, MM Institute of Medical Sciences & Research, Mullana, Haryana, IndiaDepartment of Pediatrics, MM Institute of Medical Sciences & Research, Mullana, Haryana, IndiaDepartment of Pediatrics, MM Medical College, Sadopur, Ambala, Haryana, IndiaHemophagocytic lymphohistiocytosis (HLH) is a devastating and rare multisystemic disorder characterized by an autoimmune phenomenon leading to reactive hyperactivity of cytotoxic T cells and histiocytes mediated by cytokine storm. HLH could be primary (hereditary) or secondary (acquired). Unremittent fever, organomegaly, lymphadenopathy, and neurologic dysfunction are among the common manifestations of HLH, along with abnormal lab parameters like hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and transaminitis. We report a case of a 16-year-old adolescent girl presenting to the emergency department with a history of high-grade fever, with partial response to antipyretics, body aches, and severe headache for 4 days. The physical examination revealed significant hepatomegaly. enzyme-linked immunosorbent assay was positive for dengue nonstructural protein 1 (NS1) antigen (Ag) assay. The patient was managed as per the World Health Organization (WHO) protocol and started improving from dengue; however, during the second week of the illness, the patient continued to have persistent fever. A repeat lab workup revealed bicytopenia with elevated ferritin levels (17891 ng/mL). Diagnosing dengue-associated HLH is challenging unless the treating team is aware of this association, as early recognition and timely institution of immunosuppressive and specific therapy for the underlying infection is associated with improved outcomes. Physicians should collaborate with pathologists and microbiologists for early diagnosis.https://www.pidjournal.com/doi/PID/pdf/10.5005/jp-journals-10081-1412case reportcytokine stormdengue feverdengue fever with warning signshemophagocytic lymphohistiocytosis
spellingShingle Ashwani K Sood
Saguna Pandit
Nidhi Chadha
Naresh I Jindal
Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
Pediatric Infectious Disease
case report
cytokine storm
dengue fever
dengue fever with warning signs
hemophagocytic lymphohistiocytosis
title Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
title_full Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
title_fullStr Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
title_full_unstemmed Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
title_short Secondary Hemophagocytic Lymphohistiocytosis with Severe Dengue: A Rare but Dreadful Complication
title_sort secondary hemophagocytic lymphohistiocytosis with severe dengue a rare but dreadful complication
topic case report
cytokine storm
dengue fever
dengue fever with warning signs
hemophagocytic lymphohistiocytosis
url https://www.pidjournal.com/doi/PID/pdf/10.5005/jp-journals-10081-1412
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