Hepatosplenic T-Cell Lymphoma

Jauhari, Shekeab; McKinney, Matt

doi:10.1007/978-3-030-25610-4_13

Cited by 5 publications

(8 citation statements)

References 56 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…During the second course of chemotherapy, CT examination revealed significantly reduced spleen volume (524.05 cm 3 ) while liver volume stayed similar size (1613.09 cm 3 , Fig. 1B third column), but after the third course of treatment, the spleen and liver enlarged again to 1001.58 cm 3 and 1645.19 cm 3 (Fig. 1B.…”

Section: Clinical Characteristics Of the Studied Patient With Hstclmentioning

confidence: 97%

“…1A), initially presented with fever and fatigue, and subsequently developed severe hemorrhage due to thrombocytopenia (Table . 1). CT scanning revealed splenomegaly and hepatomegaly (estimated spleen volume: 910.56 cm 3 and liver volume: 1617.21 cm 3 Fig. 1B left), while lymphadenopathy was absent (Fig.…”

Section: Clinical Characteristics Of the Studied Patient With Hstclmentioning

confidence: 99%

“…The clinical presentations of HSTCL is highly aggressive and usually with a fatal outcome. The median overall survival time is only 1-2 years [3][4][5][6] after diagnosis. Because it is rare, no treatments have become established as internationally recognized standards of care.…”

Section: Introductionmentioning

confidence: 99%

“…In addition, relapses occur often and early. In one series, the median time to relapse among patients achieving a complete response to induction therapy with CHOP or CHOP-like regimens was 3-16 months 3 . The efficient treatment modalities for HSTCL remain to be defined.…”

Section: Introductionmentioning

confidence: 99%

“…In the earliest patient series, CHOP or CHOP-like regimens were reported as the most frequently used for induction treatment. More intensive chemotherapy regimens have been used as well, such as Hyper-CVAD or Hyper-CVAD-like regimens, and ICE or IVAC regiments 3 . However, no single regimen is clearly superior [3][4][5][6] .…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

Song

Zhang

Yang

et al. 2022

Preprint

View full text Add to dashboard Cite

Hepatosplenic T-cell lymphoma (HSTCL), mostly derived from γδ T cells, is a rare but very aggressive lymphoma with poor outcomes. The molecular pathogenesis driving HSTCL is largely unknown while only limited treatment options are available. In this study, by performing paired single cell RNA-seq and T cell receptor (TCR) sequencing on biopsies from a HSTCL patient pre- and post- chemotherapy treatments, we characterized unique gene expressing signatures of malignant γδ T cells, with a set of marker genes were newly identified in HSTCL (AREG, PLEKHA5, VCAM1 etc.). Although the malignant γδ T cells were expanded from a single TCR clonotype according to their TCR identities, they evolved into two transcriptional distinct tumor subtypes during the disease progression. The Tumor_1 subtype was dominant in pre-treatment samples with highly aggressive phenotypes. While the Tumor_2 had relative mild cancer hallmark signatures but expressed genes associated with tumor survival signal and drug resistance (IL32, TOX2, AIF1, AKAP12 etc.), and finally became the main tumor subtype post-treatment. We further dissected the tumor microenvironment of the HSTCL and noticed that CD8 memory T cells were clonal expanded post-treatment. In addition, we discovered dynamically rewiring cell-cell interaction networks during the treatment. The tumor cells had reduced communications with the microenvironment post-treatment. Our study reveals heterogenous and dynamic tumor and microenvironment underlying pathogenesis of HSTCL and may contribute to identify novel targets for diagnosis and cure of HSTCL in the future.

show abstract

Section: Clinical Characteristics Of the Studied Patient With Hstclmentioning

confidence: 97%

Section: Clinical Characteristics Of the Studied Patient With Hstclmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

Song

Zhang

Yang

et al. 2022

Preprint

View full text Add to dashboard Cite

show abstract

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

et al. 2022

View full text Add to dashboard Cite

Purpose Hepatosplenic T-cell lymphoma (HSTCL), mostly derived from γδ T cells, is a rare but very aggressive lymphoma with poor outcomes. In this study, we generated the first single cell landscape for this rare disease and characterized the molecular pathogenesis underlying the disease progression. Methods We performed paired single cell RNA-seq and T cell receptor (TCR) sequencing on biopsies from a HSTCL patient pre- and post- chemotherapy treatments. Following by a series of bioinformatics analysis, we investigated the gene expression profile of γδ HSTCS as well as its tumor microenvironment (TME). Results We characterized the unique gene expressing signatures of malignant γδ T cells with a set of marker genes were newly identified in HSTCL (AREG, PLEKHA5, VCAM1 etc.). Although the malignant γδ T cells were expanded from a single TCR clonotype, they evolved into two transcriptionally distinct tumor subtypes during the disease progression. The Tumor_1 subtype was dominant in pre-treatment samples with highly aggressive phenotypes. While the Tumor_2 had relative mild cancer hallmark signatures but expressed genes associated with tumor survival signal and drug resistance (IL32, TOX2, AIF1, AKAP12, CD38 etc.), and eventually became the main tumor subtype post-treatment. We further dissected the tumor microenvironment and discovered the dynamically rewiring cell–cell interaction networks during the treatment. The tumor cells had reduced communications with the microenvironment post-treatment. Conclusions Our study reveals heterogenous and dynamic tumor and microenvironment underlying pathogenesis of HSTCL and may contribute to identify novel targets for diagnosis and treatment of HSTCL in the future.

show abstract

Case Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Disease

Tham,

Ahmad Asnawi,

Lau

et al. 2023

Case Rep Oncol

View full text Add to dashboard Cite

Hepatosplenic T-cell lymphoma is a rare form of T-cell lymphoma that predominantly emerges from neoplastic proliferation of cytotoxic T cells of γ/δ T-cell receptor-expressing lymphocytes. Isochromosome 7q and trisomy 8 are the most prevalent chromosomal abnormalities associated with hepatosplenic T-cell lymphoma, and most patients have mutations in genes related to chromatin remodeling or the JAK/STAT system. Hepatosplenic T-cell lymphoma can mimic various infectious diseases, immunological conditions, and other malignancies. Patients usually present with nonspecific constitutional symptoms and spleen and liver enlargement, with variable degrees of cytopenia. The rarity of this disease, coupled with the lack of lymph node involvement that is usually seen in lymphomas, causes significant difficulty in diagnosis, which inevitably delays the initiation of treatment. Managing this lymphoma is arduous because of its late presentation and aggressive nature, frequently resulting in rapid progression in its clinical course and refractoriness to conventional chemotherapy. There is a lack of international guidelines for its treatment, and in most cases, treatment is guided by case series. Here, we highlight the clinicopathological features and management of hepatosplenic T-cell lymphoma over a 10-year span in a single hematology referral center and review the literature.

show abstract

Hepatosplenic T-Cell Lymphoma

Cited by 5 publications

References 56 publications

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

Single cell profiling of γδ hepatosplenic T-cell lymphoma unravels tumor cell heterogeneity associated with disease progression

Case Series of Hepatosplenic T-Cell Lymphoma: A Rare and Aggressive Disease

Contact Info

Product

Resources

About