Secondary Hemophagocytic Lymphohistiocytosis Mimicking Sepsis in Adults: A Case Series.
- 1Faculty of Health Sciences, Department of Internal Medicine, Universidad Libre Barranquilla Campus, Barranquilla 08000, Colombia.
- 2Faculty of Medicine, Department of Critical Care, Universidad del Sinú Cartagena Campus, Av. El Bosque, Transversal 54 No. 30-453, Santillana, Cartagena de Indias 130015, Colombia.
- 3Faculty of Health Sciences, Department of Critical Care, Universidad de Cartagena Campus, Av. El Bosque, Transversal 54 No. 30-453, Santillana, Cartagena de Indias 130015, Colombia.
Abstract
Background and Clinical Significance: Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that can closely resemble sepsis in adults, potentially delaying recognition and treatment. Because infection may trigger or coexist with HLH, persistent or disproportionate inflammation despite appropriate antimicrobial therapy should prompt reassessment for concomitant HLH and investigation of the underlying trigger; Case Presentation: We describe three adults initially managed for presumed sepsis or severe infection at a referral hospital in Colombia. All developed persistent fever, cytopenias, organomegaly, and marked hyperferritinemia. HScore values were 222, 246, and 248, respectively; the score in Case 3 was calculated retrospectively from available pretreatment data. Bone marrow hemophagocytosis was demonstrated in Cases 1 and 2 but not in Case 3. The underlying triggers were acute myeloid leukemia, disseminated histoplasmosis in the setting of advanced HIV infection, and visceral leishmaniasis. The malignancy-associated case had an unfavorable outcome. Both infection-associated cases improved after pathogen-directed therapy combined with corticosteroids to control hyperinflammation, without escalation to an etoposide-based regimen; Conclusions: Sepsis and secondary HLH should not be regarded as mutually exclusive diagnoses. Persistent or disproportionate inflammation accompanied by progressive multilineage cytopenias, splenomegaly, markedly elevated or rising ferritin levels, hypertriglyceridemia, and/or decreasing fibrinogen should prompt early HScore calculation, assessment of HLH-2004 criteria, and concurrent investigation for an underlying trigger. Recognition of the clinical pattern and individualized treatment of both the trigger and hyperinflammatory state may facilitate timely management.
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- HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DENGUE FEVER IN A PEDIATRIC PATIENT WITH HISTORY OF AUTOIMMUNE HEMOLYTIC ANEMIA
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BibTeX
@article{guette2026secondary,
title = {{Secondary Hemophagocytic Lymphohistiocytosis Mimicking Sepsis in Adults: A Case Series.}},
author = {Damián Ochoa Guette and Jennifer Patricia Vargas Gomez and Breallan De Jesús Romero Pájaro and Rafael Tous Bertel and Amilkar Almanza Hurtado},
journal = {Reports (MDPI)},
year = {2026},
month = sep,
doi = {10.3390/reports9040329},
url = {https://europepmc.org/article/MED/42924781},
note = {CC BY},
}
RIS
TY - JOUR
TI - Secondary Hemophagocytic Lymphohistiocytosis Mimicking Sepsis in Adults: A Case Series.
AU - Damián Ochoa Guette
AU - Jennifer Patricia Vargas Gomez
AU - Breallan De Jesús Romero Pájaro
AU - Rafael Tous Bertel
AU - Amilkar Almanza Hurtado
PY - 2026
DA - 2026/09/28
JO - Reports (MDPI)
DO - 10.3390/reports9040329
UR - https://europepmc.org/article/MED/42924781
AB - Background and Clinical Significance: Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that can closely resemble sepsis in adults, potentially delaying recognition and treatment. Because infection may trigger or coexist with HLH, persistent or disproportionate inflammation despite appropriate antimicrobial therapy should prompt reassessment for concomitant HLH and investigation of the underlying trigger; Case Presentation: We describe three adults initially managed for presumed sepsis or severe infection at a referral hospital in Colombia. All developed persistent fever, cytopenias, organomegaly, and marked hyperferritinemia. HScore values were 222, 246, and 248, respectively; the score in Case 3 was calculated retrospectively from available pretreatment data. Bone marrow hemophagocytosis was demonstrated in Cases 1 and 2 but not in Case 3. The underlying triggers were acute myeloid leukemia, disseminated histoplasmosis in the setting of advanced HIV infection, and visceral leishmaniasis. The malignancy-associated case had an unfavorable outcome. Both infection-associated cases improved after pathogen-directed therapy combined with corticosteroids to control hyperinflammation, without escalation to an etoposide-based regimen; Conclusions: Sepsis and secondary HLH should not be regarded as mutually exclusive diagnoses. Persistent or disproportionate inflammation accompanied by progressive multilineage cytopenias, splenomegaly, markedly elevated or rising ferritin levels, hypertriglyceridemia, and/or decreasing fibrinogen should prompt early HScore calculation, assessment of HLH-2004 criteria, and concurrent investigation for an underlying trigger. Recognition of the clinical pattern and individualized treatment of both the trigger and hyperinflammatory state may facilitate timely management.
SN - 2571-841X
ER -