Elevated monocyte HLA-DR in pediatric secondary hemophagocytic lymphohistiocytosis : a retrospective study
Copyright © 2023 Raimbault, Monneret, Gossez, Venet, Belot, Zekre, Remy and Javouhey..
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition, and its diagnosis may be challenging. In particular, some cases show close similarities to sepsis (fever, organ failure, and high ferritin), but their treatment, while urgent, differ: prompt broad-spectrum antibiotherapy for sepsis and immunosuppressive treatment for HLH. We questioned whether monocyte human leucocyte antigen (mHLA)-DR could be a diagnostic marker for secondary HLH (sHLH).
Methods: We retrospectively reviewed data from patients with a sHLH diagnosis and mHLA-DR quantification. mHLA-DR data from healthy children and children with septic shock, whose HLA-DR expression is reduced, from a previously published study were also included for comparison.
Results: Six patients with sHLH had mHLA-DR quantification. The median level of monocyte mHLA-DR expression in patients with sHLH [79,409 antibodies bound per cell (AB/C), interquartile range (IQR) (75,734-86,453)] was significantly higher than that in healthy children and those with septic shock (29,668 AB/C, IQR (24,335-39,199), and 7,493 AB/C, IQR (3,758-14,659), respectively). Each patient with sHLH had a mHLA-DR higher than our laboratory normal values. Four patients had a second mHLA-DR sampling 2 to 4 days after the initial analysis and treatment initiation with high-dose corticosteroids; for all patients, mHLA-DR decreased to within or close to the normal range. One patient with systemic juvenile idiopathic arthritis had repeated mHLA-DR measurements over a 200-day period during which she underwent four HLH episodes. mHLA-DR increased during relapses and normalized after treatment incrementation.
Conclusion: In this small series, mHLA-DR was systematically elevated in patients with sHLH. Elevated mHLA-DR could contribute to sHLH diagnosis and help earlier distinction with septic shock.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:14 |
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| Enthalten in: |
Frontiers in immunology - 14(2023) vom: 24., Seite 1286749 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Raimbault, Sylvain [VerfasserIn] |
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| Links: |
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| Themen: |
Cytokine storm |
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| Anmerkungen: |
Date Completed 16.12.2023 Date Revised 27.03.2024 published: Electronic-eCollection Citation Status MEDLINE |
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| doi: |
10.3389/fimmu.2023.1286749 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM365681342 |
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| 100 | 1 | |a Raimbault, Sylvain |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Elevated monocyte HLA-DR in pediatric secondary hemophagocytic lymphohistiocytosis |b a retrospective study |
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| 500 | |a Date Revised 27.03.2024 | ||
| 500 | |a published: Electronic-eCollection | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023 Raimbault, Monneret, Gossez, Venet, Belot, Zekre, Remy and Javouhey. | ||
| 520 | |a Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition, and its diagnosis may be challenging. In particular, some cases show close similarities to sepsis (fever, organ failure, and high ferritin), but their treatment, while urgent, differ: prompt broad-spectrum antibiotherapy for sepsis and immunosuppressive treatment for HLH. We questioned whether monocyte human leucocyte antigen (mHLA)-DR could be a diagnostic marker for secondary HLH (sHLH) | ||
| 520 | |a Methods: We retrospectively reviewed data from patients with a sHLH diagnosis and mHLA-DR quantification. mHLA-DR data from healthy children and children with septic shock, whose HLA-DR expression is reduced, from a previously published study were also included for comparison | ||
| 520 | |a Results: Six patients with sHLH had mHLA-DR quantification. The median level of monocyte mHLA-DR expression in patients with sHLH [79,409 antibodies bound per cell (AB/C), interquartile range (IQR) (75,734-86,453)] was significantly higher than that in healthy children and those with septic shock (29,668 AB/C, IQR (24,335-39,199), and 7,493 AB/C, IQR (3,758-14,659), respectively). Each patient with sHLH had a mHLA-DR higher than our laboratory normal values. Four patients had a second mHLA-DR sampling 2 to 4 days after the initial analysis and treatment initiation with high-dose corticosteroids; for all patients, mHLA-DR decreased to within or close to the normal range. One patient with systemic juvenile idiopathic arthritis had repeated mHLA-DR measurements over a 200-day period during which she underwent four HLH episodes. mHLA-DR increased during relapses and normalized after treatment incrementation | ||
| 520 | |a Conclusion: In this small series, mHLA-DR was systematically elevated in patients with sHLH. Elevated mHLA-DR could contribute to sHLH diagnosis and help earlier distinction with septic shock | ||
| 650 | 4 | |a Journal Article | |
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| 650 | 4 | |a cytokine storm | |
| 650 | 4 | |a hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a lymphocyte activation | |
| 650 | 4 | |a macrophage activation syndrome | |
| 650 | 4 | |a monocyte HLA-DR | |
| 650 | 7 | |a HLA-DR Antigens |2 NLM | |
| 700 | 1 | |a Monneret, Guillaume |e verfasserin |4 aut | |
| 700 | 1 | |a Gossez, Morgane |e verfasserin |4 aut | |
| 700 | 1 | |a Venet, Fabienne |e verfasserin |4 aut | |
| 700 | 1 | |a Belot, Alexandre |e verfasserin |4 aut | |
| 700 | 1 | |a Zekre, Franck |e verfasserin |4 aut | |
| 700 | 1 | |a Remy, Solene |e verfasserin |4 aut | |
| 700 | 1 | |a Javouhey, Etienne |e verfasserin |4 aut | |
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