Pediatric Hepatosplenic T-cell lymphoma
2023-06-25 Sheng Xiao, MD , Chunxiao Yang Affiliation1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
2. Brigham and Women's Hospital, Harvard Medical School, Boston , MA (USA)
Classification
Definition
Pediatric hepatosplenic T-cell lymphoma (pHSTCL) is an aggressive disease characterized by the malignant proliferation of T-cells in the liver and spleen. Unlike other lymphomas, pHSTCL rarely involves lymph nodes or other organs.1 i(7)(q10) is a common finding in pHSTCL, leading to a gain of 7q including ABCB1 at 7q22.2,3 Trisomy 8 is also seen in some cases. Mutations of SETD2, STAT5B, INO80, TET3, SMARCA2, STAT3 are frequently observed.4,5 These tumors mostly express TCR γ/δ.1
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 32756940 | 2020 | Hepatosplenic T-cell lymphoma: a rare but challenging entity. | Pro B et al |
| 2 | 11807981 | 2002 | Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression. | Wlodarska I et al |
| 3 | 25057852 | 2014 | Integrative genomic and transcriptomic analysis identified candidate genes implicated in the pathogenesis of hepatosplenic T-cell lymphoma. | Finalet Ferreiro J et al |
| 4 | 24947020 | 2014 | Frequent STAT5B mutations in γδ hepatosplenic T-cell lymphomas. | Nicolae A et al |
| 5 | 28122867 | 2017 | The Genetic Basis of Hepatosplenic T-cell Lymphoma. | McKinney M et al |
Citation
Sheng Xiao, MD ; Chunxiao Yang
Pediatric Hepatosplenic T-cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2023-06-25
Online version: http://atlasgeneticsoncology.org/solid-tumor/209171/pediatric-hepatosplenic-t-cell-lymphoma
