Importance of Early Recognition and Initiation of Management in Haemophagocytic Lymphohistiocytosis: A Case Report

We present the case of an 18-year-old previously healthy male who was admitted with severe abdominal pain and subsequently found to have concurrent Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infections. Due to concern over potential deterioration, he was transferred to critical care, where progressive cytopenias and deranged liver function tests were noted. The combination of acute liver failure, pancytopenia, hyperferritinaemia, and coagulopathy raised suspicion for haemophagocytic lymphohistiocytosis (HLH). This was supported by bone marrow aspirate findings demonstrating megakaryocytic proliferation and elevated soluble CD25 levels on further testing performed at the Great Ormond Street Hospital (GOSH). High-dose methylprednisolone and anakinra were initiated with subsequent clinical and biochemical improvement. Targeted antiviral therapy with ganciclovir and rituximab was commenced for CMV and EBV, respectively. The patient's haematological parameters and liver function were normalised, and he was discharged on a tapering regimen of prednisolone and anakinra, alongside oral valganciclovir. This case highlights the importance of early recognition of HLH in the context of viral coinfection, particularly EBV and CMV. Prompt multidisciplinary involvement and initiation of immunosuppressive and antiviral therapy are crucial to improving outcomes in this potentially fatal condition.

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Publication Details

Journal
Cureus
Published
2025-11-24
DOI
https://doi.org/10.7759/cureus.97645
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
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0.00
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article

Importance of Early Recognition and Initiation of Management in Haemophagocytic Lymphohistiocytosis: A Case Report

Sarah Jones, Sreemallika Ramireddy
Cureus
Autoimmune and Inflammatory Disorders Research
article

Importance of Early Recognition and Initiation of Management in Haemophagocytic Lymphohistiocytosis: A Case Report

Sarah Jones, Sreemallika Ramireddy
article en

Abstract

We present the case of an 18-year-old previously healthy male who was admitted with severe abdominal pain and subsequently found to have concurrent Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infections. Due to concern over potential deterioration, he was transferred to critical care, where progressive cytopenias and deranged liver function tests were noted. The combination of acute liver failure, pancytopenia, hyperferritinaemia, and coagulopathy raised suspicion for haemophagocytic lymphohistiocytosis (HLH). This was supported by bone marrow aspirate findings demonstrating megakaryocytic proliferation and elevated soluble CD25 levels on further testing performed at the Great Ormond Street Hospital (GOSH). High-dose methylprednisolone and anakinra were initiated with subsequent clinical and biochemical improvement. Targeted antiviral therapy with ganciclovir and rituximab was commenced for CMV and EBV, respectively. The patient's haematological parameters and liver function were normalised, and he was discharged on a tapering regimen of prednisolone and anakinra, alongside oral valganciclovir. This case highlights the importance of early recognition of HLH in the context of viral coinfection, particularly EBV and CMV. Prompt multidisciplinary involvement and initiation of immunosuppressive and antiviral therapy are crucial to improving outcomes in this potentially fatal condition.

CureusVol. 17(11)
Aneurin Bevan University Health Board (GB)
Openalex Percentile: Top 53%
Autoimmune and Inflammatory Disorders Research
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