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European Radiology

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25-08-2023 | Hemophagocytic Lymphohistiocytosis | Magnetic Resonance

Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis

Authors: Wei Ma, Liang Zhou, Wei Li, Xiujuan Li, Yan Huang, Sijie Gao, Jie Yu, Yuan Fang, Ye Xu

Published in: European Radiology | Issue 2/2024

Abstract

Objectives

To investigate the association of brain MRI and clinical variables with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis (CNS-HLH).

Methods

Clinical and brain MRI data of children with CNS-HLH from January 2012 to March 2022 were reviewed retrospectively. Patients were divided into the deceased group and the surviving group. The intergroup differences of seven brain MRI variables, twelve clinical variables, and underlying diseases were studied.

Results

One hundred and fourteen patients were included in this study, consisting of 59 who died and 55 who survived. The included clinical variables did not show statistically independent correlation with patients’ deaths. For MRI variables, a multivariate analysis demonstrated restricted diffusion of lesion (OR = 9.64, 95% CI: 3.39–27.43, p < 0.001) and count of affected brain regions (CABR) (OR = 1.24, 95% CI: 1.03–1.49, p = 0.02) were independent risk factors for death. ROC curve showed CABR (AUC = 0.79, 95% CI: 0.70–0.87, p < 0.001) is highly predictive for mortality with an optimal cutoff value of 4.5 (sensitivity 76%, specificity 73%). For HLH subtypes, familial HLH (F-HLH, OR = 9.90, 95% CI: 2.01–48.87, p = 0.005) and immune-compromise-related HLH (IC-HLH, OR = 4.95, 95% CI: 1.40–17.46, p = 0.01) presented statistically stronger association with death than infection-related HLH. F-HLH and IC-HLH preferred to have large lesions, restricted diffusion, and more brain regions involved than other subtypes.

Conclusion

Brain MRI features exhibit independent prediction for mortality in children with CNS-HLH, and HLH subtypes pose effects on patient outcomes and brain MRI findings.

Clinical relevance statement

The number of affected brain regions and diffusion restriction of lesion exhibit significant correlation with mortality in children diagnosed with CNS-hemophagocytic lymphohistiocytosis, and may serve as candidate MRI markers for the prediction of the disorder’s severity.

Key Points

• The brain MRI markers, restricted diffusion of lesion and count of affected brain regions, significantly correlated with death.

• Familial and immune-compromise-related hemophagocytic lymphohistiocytosis presented statistically stronger association with death than infection-related subtype.

• Brain MRI is potential in death-predicting for children with central nervous system involvement of hemophagocytic lymphohistiocytosis.

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Title: Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis
Authors: Wei Ma
Liang Zhou
Wei Li
Xiujuan Li
Yan Huang
Sijie Gao
Jie Yu
Yuan Fang
Ye Xu
Publication date: 25-08-2023
Publisher: Springer Berlin Heidelberg
Keywords: Hemophagocytic Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis
Cranial MRI
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Published in: European Radiology / Issue 2/2024
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI: https://doi.org/10.1007/s00330-023-10147-8

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis

Abstract

Objectives

Methods

Results

Conclusion

Clinical relevance statement

Key Points

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusion

Clinical relevance statement

Key Points

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