Imaging Phenotypes and Evolution of Hepatic Langerhans Cell Histiocytosis on CT/MRI: A Retrospective Study of Clinical Cases and Literature Review
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. CT and MRI Examinations
2.3. Image Analysis
2.4. Statistical Analysis
3. Results
3.1. Patients and Clinical Characteristics
3.2. Image Phenotypes Based on Lesion Distribution
3.3. Detailed Imaging Findings and Evolution of Lesions
3.3.1. Parenchymal Lesions
- Evolution of parenchymal lesions in eight patients with multiple MRI scans
3.3.2. Periportal Abnormalities
- Evolution of periportal lesions in eight patients with multiple MRI scans
3.3.3. Hepatic Fibrosis and Other Imaging Findings
4. Discussion
4.1. Differences among the Three Imaging Phenotypes: Patient Age, Clinical Feature, and Fibrosis
- Central periportal lesion phenotype
- Disseminated lesion phenotype
- Scattered lesion phenotype
4.2. Evolution of Lesions
4.3. Detailed Additions to Imaging Findings
4.4. Earlier Detection of Liver Fibrosis
4.5. Limitations
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Characteristics | Combined a | p-Value 1 b | Reference Reported c | Present Study | Scattered Lesion Type in Present Study | Disseminated Lesion Type in Present Study | Central Periportal Lesion Type in Present Study | p-Value 2 d |
---|---|---|---|---|---|---|---|---|
Number of patients | 87 | 67 | 20 | 6 | 8 | 6 | ||
Sex, n (%) | 0.851 | 1.000 | ||||||
Male | 55 (63.2) | 42 (62.7) | 13 (65) | 4 (66.7) | 5 (62.5) | 4 (66.7) | ||
Female | 32 (36.8) | 25 (37.3) | 7 (35) | 2 (33.3) | 3 (37.5) | 2 (33.3) | ||
Age at diagnosis, n (%) | 0.620 | <0.001 * | ||||||
≥15 Y | 52 (59.8) | 41 (61.2) | 11 (55) | 6 (100) | 5 (62.5) | 0 | ||
<15 Y | 35 (40.2) | 26 (38.8) | 9 (45) | 0 | 3 (37.5) | 6 (100) | ||
Mean ± SD, (Years) | NA | NA | 19.5 ± 16.5 | 36.8 ± 8.4 | 19.7 ± 12.9 | 1.8 ± 0.4 | ||
Range, (Years) | NA | NA | 1.1~53 | 29~53 | 1.1~39 | 1.2~2.5 | ||
Liver involvement, n(%) | / | / | ||||||
At baseline | NA | NA | 18 (90) | 4 (66.7) | 8 (100) | 6 (100) | ||
NA | NA | NA | 2 (10) | 2 (33.3) | 0 | 0 | ||
Hepatomegaly, n (%) | 42 (62.7) | 0.798 | 29 (61.7) e | 13 (65.0) | 1 (16.7) | 7 (87.5) | 5 (83.3) | 0.019 * |
Liver biochemical abnormalities, n (%) | 54 (74.0) | 0.471 | 38 (71.7) f | 16 (80.0) | 2 (33.3) | 8 (100.0) | 6 (100.0) | 0.006 * |
Stratification, n (%) | 1.000 | 1.000 | ||||||
MS-LCH | 78 (96.3) | 59 (96.7) g | 19 (95) | 6 (100) | 7 (87.5) | 6 (100) | ||
SS-LCH | 3 (3.7) | 2 (3.3) | 1 (5) | 0 | 1 (12.5) | 0 | ||
Other organ, n (%) | / | 0.004 * | ||||||
Bones | NA | NA | 10 (50) | 5 (83.3) | 2 (25) | 3 (50) | ||
Lung | NA | NA | 8 (40) | 2 (33.3) | 5 (62.5) | 1 (16.7) | ||
Skin | NA | NA | 8 (40) | 0 | 3 (37.5) | 5 (83.3) | ||
Lymph node | NA | NA | 4 (20) | 1 (16.7) | 0 | 3 (50) | ||
pituitary | NA | NA | 6 (30) | 1 (16.7) | 5 (62.5) | 0 | ||
Thymus | NA | NA | 1 (5) | 0 | 1 (12.5) | 0 | ||
Thyroid | NA | NA | 3 (15) | 1 (16.7) | 2 (25) | 0 | ||
bone marrow | NA | NA | 2 (10) | 0 | 1 (12.5) | 1 (16.7) | ||
Mucosa | NA | NA | 3 (15) | 0 | 0 | 3 (50) | ||
Nervous system | NA | NA | 2 (10) | 1 (16.7) | 1 (12.5) | 0 | ||
Ear | NA | NA | 2 (10) | 0 | 0 | 2 (33.3) | ||
Genitalia | NA | NA | 1 (5) | 0 | 1 (12.5) | 0 | ||
Spleen | NA | NA | 3 (15) | 0 | 1 (12.5) | 2 (33.3) |
Liver Biochemical Results | Scattered Lesion Phenotype | Disseminated Lesion Phenotype | Central Periportal Lesion Phenotype | p-Value |
---|---|---|---|---|
Number of patients | 6 | 8 | 6 | |
γGT, AP | <0.001 * | |||
Normal | 4 | 0 | 0 | |
γGT increased ≤2 times normal/AP increased ≤1.5 times normal | 2 | 2 | 0 | |
γGT increased >2 times normal/AP increased >1.5 times normal | 0 | 6 | 6 | |
ALT, AST | 0.025 * | |||
Normal | 5 | 2 | 0 | |
ALT/AST increased ≤3 times normal | 1 | 4 | 1 | |
3 times normal <ALT/AST increased ≤5 times normal | 0 | 1 | 2 | |
ALT/AST increased >5 times normal | 0 | 1 | 3 | |
Albumin | 0.066 | |||
Normal | 6 | 6 | 2 | |
Hypoalbuminemia >30 g/dl | 0 | 2 | 4 | |
Hypoalbuminemia <30 g/dl | 0 | 0 | 0 | |
Bilirubin | 0.01 * | |||
Normal | 6 | 6 | 1 | |
Hyperbilirubinemia ≤3 times normal | 0 | 1 | 4 | |
3 times normal < hyperbilirubinemia ≤5 times normal | 0 | 1 | 0 | |
Hyperbilirubinemia >5 times normal | 0 | 0 | 1 |
Accompanied Imaging Signs | All Patients | Scattered Lesion Type | Disseminated Lesion Type | Central Periportal Lesion Type | p Value b |
---|---|---|---|---|---|
Number of patients | 20 | 6 | 8 | 6 | |
T2/DWI hypointense, n (%) | 0.019 * | ||||
Periportal halo sign | 2 (13) | 0 | 0 | 2 (33.3) | |
Giant, central nodular fibrosis | 1 (6.7) | 0 | 0 | 1 (16.7) | |
Patchy fibrosis | 6 (40) | 0 | 3 (60) | 3 (50) | |
NA a | 5 (25) | 2 (33.3) | 3 (37.5) | 0 | |
Hepatic surface, n (%) | 0.035 * | ||||
Smooth | 17 (85) | 6 (100) | 8 (100) | 3 (50) | |
Nodular | 3 (15) | 0 | 0 | 3 (50) | |
Hepatomegaly, n (%) | 13 (65) | 1 (16.7) | 7 (87.5) | 5 (83.3) | 0.019 * |
Splenomegaly, n (%) | 12 (60) | 1 (16.7) | 6 (75) | 5 (83.3) | 0.053 |
Enlarged lymph Nodes, n (%) | 11 (55) | 0 | 7 (87.5) | 4 (66.7) | 0.005 * |
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Hao, L.; Li, Y.; Xiong, Z.; Jiang, Y.; Hu, X.; Hu, D.; Li, Z.; Shen, Y. Imaging Phenotypes and Evolution of Hepatic Langerhans Cell Histiocytosis on CT/MRI: A Retrospective Study of Clinical Cases and Literature Review. Bioengineering 2023, 10, 598. https://doi.org/10.3390/bioengineering10050598
Hao L, Li Y, Xiong Z, Jiang Y, Hu X, Hu D, Li Z, Shen Y. Imaging Phenotypes and Evolution of Hepatic Langerhans Cell Histiocytosis on CT/MRI: A Retrospective Study of Clinical Cases and Literature Review. Bioengineering. 2023; 10(5):598. https://doi.org/10.3390/bioengineering10050598
Chicago/Turabian StyleHao, Luwen, Yuanqiu Li, Ziman Xiong, Yuchen Jiang, Xuemei Hu, Daoyu Hu, Zhen Li, and Yaqi Shen. 2023. "Imaging Phenotypes and Evolution of Hepatic Langerhans Cell Histiocytosis on CT/MRI: A Retrospective Study of Clinical Cases and Literature Review" Bioengineering 10, no. 5: 598. https://doi.org/10.3390/bioengineering10050598
APA StyleHao, L., Li, Y., Xiong, Z., Jiang, Y., Hu, X., Hu, D., Li, Z., & Shen, Y. (2023). Imaging Phenotypes and Evolution of Hepatic Langerhans Cell Histiocytosis on CT/MRI: A Retrospective Study of Clinical Cases and Literature Review. Bioengineering, 10(5), 598. https://doi.org/10.3390/bioengineering10050598