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Research Article

Comprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis

, , , , & ORCID Icon
Article: 2342276 | Received 20 Nov 2023, Accepted 08 Apr 2024, Published online: 17 Apr 2024
 

ABSTRACT

Host immune dysfunction plays a crucial role in the onset, progression, and outcome of hemophagocytic lymphohistiocytosis (HLH). This study aimed to comprehensively evaluate the peripheral immune profiles in patients with newly diagnosed secondary hemophagocytic lymphohistiocytosis (sHLH), and explore their predictive value for patient prognosis. A total of 77 patients with sHLH were enrolled in this study, with 31 of them experiencing mortality. Flow cytometry was used to assess the percentages, absolute numbers, and phenotypes of lymphocyte subsets. Simultaneously, cytokine levels and routine laboratory indicators were also collected. In sHLH patients, lymphocyte subset absolute numbers were significantly impaired, accompanied by T cell hyperactivation, B cell hyperactivation, and increased plasmablast proliferation. Prognostic analysis revealed that lower CD8+ T cell percentages, elevated APTT, IL-6, IL-10 levels, and increased CD4+CD28null T cell proportions were associated with poor patient outcomes. The study demonstrates dysregulation in the counts and phenotypes of lymphocyte subsets in sHLH patients. Several key factors, including IL-6, IL-10, APTT, and various T cell percentages, have potential as prognostic markers and therapeutic targets in sHLH.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Authors’ contributions

YW and SW wrote the manuscript. XY, TW, WW collected the materials of patients. YW did the flow cytometry detection. HH and SW analysed the data and helped revise the manuscript.

Availability of data and material

All data generated or analysed during this study have been deposited in Figshare with DOI 10.6084/m9.figshare.24572764.

Consent for participate

Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin. Written informed consent was obtained from the individual(s), and minor(s)’ legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.

Consent for publication

All authors contributed to the article and approved the submitted version.

Supplemental data

Supplemental data for this article can be accessed online at https://doi.org/10.1080/21505594.2024.2342276.