Indian Journal of Critical Care Case Report

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VOLUME 3 , ISSUE 6 ( November-December, 2024 ) > List of Articles

CASE SERIES

Enlightening Role of Intravenous Immunoglobulin in Secondary Hemophagocytic Lymphohistiocytosis with Sepsis in an Intensive Care Unit

Krishnkant Bhardwaj, Gowthaman Thatta Balakrishnan, Pradeep Moolchandani, Saurabh Saigal, Rajesh Panda, Abhijeet Anand, Saiteja Kodamanchili, Puru Prajapati

Keywords : Hemophagocytic lymphohistiocytosis, Hemophagocytic lymphohistiocytosis, Intensive care unit, Intravenous immunoglobulin, Sepsis

Citation Information : Bhardwaj K, Balakrishnan GT, Moolchandani P, Saigal S, Panda R, Anand A, Kodamanchili S, Prajapati P. Enlightening Role of Intravenous Immunoglobulin in Secondary Hemophagocytic Lymphohistiocytosis with Sepsis in an Intensive Care Unit. 2024; 3 (6):154-156.

DOI: 10.5005/jp-journals-11006-0133

License: CC BY-NC 4.0

Published Online: 19-10-2024

Copyright Statement:  Copyright © 2024; The Author(s).


Abstract

Aim and background: Hemophagocytic lymphohistiocytosis (HLH) presents a challenge due to its catastrophic nature of uncontrolled immune activation and nonspecific symptoms, complicating diagnosis. Despite its association with poor outcomes, guidelines recommend multimodal approaches including intravenous immunoglobulin (IVIG). In this case series, we highlight the role of IVIG in improving outcomes. Case description: We illustrate the clinical courses of four patients with secondary HLH. All cases fulfilled diagnostic criteria for HLH, exhibiting fever, elevated ferritin levels, cell lineage abnormalities, and hemophagocytosis on bone marrow biopsy. All four patients required high doses of vasopressors. IVIG (2 gm/kg over 3 days) was initiated at various stages of treatment alongside dexamethasone 10 mg/m2/day. Two patients who received early IVIG demonstrated improvements in cell lineage and hemodynamics, while the other two, receiving IVIG relatively late due to delayed manifestations or diagnoses, succumbed to their illness. Conclusion: Diagnosing HLH is challenging due to its nonspecific features, and the presence of sepsis and septic shock in intensive care settings further complicates diagnosis. We recommend early evaluation of patients with a high suspicion of HLH, with prompt bone marrow biopsy. We observed clinical and laboratory improvements following initiation of IVIG therapy (2 gm/kg over 3 days) alongside dexamethasone. Clinical significance: This case series emphasizes the importance of early HLH diagnosis and the initiation of a short course of IVIG (2 gm/kg over 3 days) as the preferred initial treatment, along with dexamethasone, rather than as a salvage therapy.


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