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Review
. 2025 Feb 18;11(2):158.
doi: 10.3390/jof11020158.

Fungal-Induced Hemophagocytic Lymphohistiocytosis: A Literature Review in Non-HIV Populations

Chia-Yu Chiu  1 , Rachel S Hicklen  2 , Dimitrios P Kontoyiannis  3
Affiliations
Review

Fungal-Induced Hemophagocytic Lymphohistiocytosis: A Literature Review in Non-HIV Populations

Chia-Yu Chiu et al. J Fungi (Basel). .

Abstract

We performed a thorough search of the literature published through December 2024 with no date exclusions on invasive fungal infection (IFI)-induced hemophagocytic lymphohistiocytosis (HLH) in non-human immunodeficiency virus (HIV) patients. The frequency of IFI-induced HLH reported across 16 articles was 9%. Of the 116 identified cases with available clinical information, 53% occurred in immunocompromised patients. IFIs were usually disseminated (76%), with Histoplasma capsulatum being the most common pathogen (51%). IFI and HLH were diagnosed simultaneously in most cases (78%). The 30-day survival rate was 64%. Reported cases had significant heterogeneity in patient characteristics, management strategies, and outcomes.

Keywords: hematologic malignancies; hemophagocytic lymphohistiocytosis; immunocompromised hosts; invasive fungal infection; solid organ transplant.

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Conflict of interest statement

D.P.K. reports honoraria and research support from Gilead Sciences and Astellas Pharma. He received consultant fees from Astellas Pharma, Merck, and Gilead Sciences and is a member of the Data Review Committee of Cidara Therapeutics, AbbVie, Scynexis, and the Mycoses Study Group.

Figures

Figure 1
Figure 1
Flow diagram of the literature review methodology used to identify all reported cases of fungal-induced HLH in non-HIV patients, with no date restriction applied until December 2024. Abbreviations: HIV, human immunodeficiency virus; HLH, hemophagocytic lymphohistiocytosis.
See this image and copyright information in PMC

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References

    1. Cox M.F., Mackenzie S., Low R., Brown M., Sanchez E., Carr A., Carpenter B., Bishton M., Duncombe A., Akpabio A., et al. Diagnosis and Investigation of Suspected Haemophagocytic Lymphohistiocytosis in Adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) Consensus Guideline. Lancet Rheumatol. 2024;6:e51–e62. doi: 10.1016/S2665-9913(23)00273-4. - DOI - PubMed
    1. Ramos-Casals M., Brito-Zerón P., López-Guillermo A., Khamashta M.A., Bosch X. Adult Haemophagocytic Syndrome. Lancet. 2014;383:1503–1516. doi: 10.1016/S0140-6736(13)61048-X. - DOI - PubMed
    1. Henter J.-I., Horne A., Aricó M., Egeler R.M., Filipovich A.H., Imashuku S., Ladisch S., McClain K., Webb D., Winiarski J., et al. HLH-2004: Diagnostic and Therapeutic Guidelines for Hemophagocytic Lymphohistiocytosis. Pediatr. Blood Cancer. 2007;48:124–131. doi: 10.1002/pbc.21039. - DOI - PubMed
    1. Abdelhay A., Mahmoud A.A., Al Ali O., Hashem A., Orakzai A., Jamshed S. Epidemiology, Characteristics, and Outcomes of Adult Haemophagocytic Lymphohistiocytosis in the USA, 2006–2019: A National, Retrospective Cohort Study. EClinicalMedicine. 2023;62:102143. doi: 10.1016/j.eclinm.2023.102143. - DOI - PMC - PubMed
    1. Townsend J.L., Shanbhag S., Hancock J., Bowman K., Nijhawan A.E. Histoplasmosis-Induced Hemophagocytic Syndrome: A Case Series and Review of the Literature. Open Forum Infect. Dis. 2015;2:ofv055. doi: 10.1093/ofid/ofv055. - DOI - PMC - PubMed

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