Hepatosplenic γδ T-cell Lymphoma: An Overview

Visnyei, Koppany; Grossbard, Michael L.; Shapira, Ilan

doi:10.1016/j.clml.2013.03.011

Cited by 30 publications

(33 citation statements)

References 111 publications

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“…For patients with HSTCL, there is no standard of care. Outcomes are poor with conventional chemotherapy approaches . Purine analogs have demonstrated activity and consolidation with allogeneic stem cell transplant has been curative in up to 41% of patients after a CHOP like regimen.…”

Section: Discussionmentioning

confidence: 99%

Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas

et al. 2019

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The T Cell Project was the largest prospective trial to explore the incidence, treatment patterns, and outcomes for T cell lymphomas. The rare subtypes of T cell lymphomas, including hepatosplenic T cell lymphoma (HSTCL), enteropathy associated T cell lymphoma (EATL), and peripheral gamma delta T cell lymphomas (PGDTCLs) are poorly represented in most studies and there is little data regarding treatment patterns. We report results from 115 patients with hepatosplenic (n = 31), enteropathy associated (n = 65), and PGDTCLs (n = 19). While anthracycline regimens were most commonly used as first line therapy, response rates ranged from 20%-40% and were suboptimal for all groups. Autologous stem cell transplantation was performed as a consolidation in first remission in a small number of patients (33% of HSTCL, 7% of EATL, and 12% of PGDTCL), and four patients with HSTCL underwent allogeneic stem cell transplantation in first remission. The progression free survival at 3 years ranged from 28%-40% for these rare subtypes, and the overall survival at 3 years was

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Section: Discussionmentioning

confidence: 99%

Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas

et al. 2019

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“…HSTL cells usually derive from a functionally immature cytotoxic γδ T cell,and usually occurs in adolescents and young adults(68%) [19]. Patients initially present with fever, fatigue, weight loss, and abdominal discomfort due to splenomegaly and hepatomegaly.…”

Section: Discussionmentioning

confidence: 99%

Primary spleen extranodal NK/T cell lymphoma, nasal type, with bone marrow involvement and CD30 positive expression: a case report and literature review

Cao

Huang

et al. 2014

Diagn Pathol

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AimsPrimay spleen NK/T cell lymphoma is very rare. We report a case of 39-years-old male of primary splenic NK/T cell lymphoma with bone marrow involvement and CD30 positive expression.Case descriptionThe patient had high fever for 2 months, and CT scan revealed a diffuse splenomegaly without hepatomegaly. The diagnosis was established by splenectomy specimen and bone marrow biopsy. Normal spleen structure was destroyed by the diffusely infiltrated neoplastic cells, and one of the splenic hilar lymph nodes was involved. The lymphomatous cells were mainly medium-sized, mixed with small and large cells with pleomorphic nuclei and conspicuous nucleoli. Angiocentric growth pattern was present, with mitotic figures and apoptotic bodies easily being found. These neoplastic cells demonstrated a typical immunophenotype of CD2, CD3ε, CD7, CD4, CD56, TIA-1, Granzyme B, CD30 positive, and CD5, CD8, CD20, CD79a negative. The Epstein-Barr virus encoded RNAs (EBERs) genomes were also found in tumor cells by in situ hybridization, while no clonal rearrangement of the T cell receptor-γ genes (TCRG) was found. Biopsy of bone marrow revealed scattered atypical cells presented with a predominantly intrasinusoidal distribution. A diagnosis as primary spleen NK/T cell lymphoma, nasal type (ENKTL) with CD30 expression and bone marrow involvement was finally made. The patient received chemotherapy and was still alive 6 months after splenectomy.Clinical significancePrimary spleen ENKTL is very rare, it should be made with the combination of clinical feature, PET-CT image, and pathological characteristics, and should be distinguished from other lymphomas or leukemia involved in spleen.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_169

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“…HSTCL is a highly aggressive lymphoma showing a poor prognosis and resistance to conventional therapies [6] . From International Peripheral T-Cell and Natural Killer/T-Cell Lymphoma Study, 5-year failure-free and overall survival were 0% and 7%, respectively which was the worst among all subtypes of T-cell lymphomas [1] .…”

Section: Discussionmentioning

confidence: 99%

“…HSTCL is usually composed of medium-sized lymphoid cells with marked sinusoidal infiltration of spleen, liver and bone marrow [5] . This subtype of lymphoma is more common in young men who are often presented with B-symptoms, hepatosplenomegaly, cytopenia without significant lymphadenopathy and have poor outcomes [6] . The authors report two additional cases of HSTCL from Maharaj Nakorn Chiang-Mai Hospital in Thailand.…”

Section: Introductionmentioning

confidence: 99%

Hepatosplenic T-cell lymphoma: Report of two cases

Rattarittamrong

Norrasethada

Ya-In

et al. 2014

CRIM

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Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of lymphoma. Presenting with hepatosplenomegaly, fever, cytopenia without significant lymphadenopathy, most patients are young men with poor outcomes. Here is the report of two cases of HSTCL from Maharaj Nakorn Chiang-Mai Hospital, Thailand. Both patients were middle-aged men presented with prolonged fever, hepatosplenomegaly and cytopenia. Abnormal lymphoid cells, not demonstrated by flow cytometry, were microscopically revealed in the patients’ bone marrow. The diagnosis of HSTCL was based on the histopathologic section obtained from splenectomy and liver biopsy. Both patients received cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) regimen initially while one of them was salvaged with etoposide, methylprednisolone, cytarabine, cisplatin (ESHAP) and methotrexate, high-dose cytarabine, methylprednisolone (cycle B of hyperCVAD) regimen. They responded poorly to chemotherapy and succumbed to severe sepsis. The presented cases confirmed that HSTCL is very difficult to diagnose and current treatment modalities appear to be ineffective

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Hepatosplenic γδ T-cell Lymphoma: An Overview

Cited by 30 publications

References 111 publications

Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas

Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas

Primary spleen extranodal NK/T cell lymphoma, nasal type, with bone marrow involvement and CD30 positive expression: a case report and literature review

Hepatosplenic T-cell lymphoma: Report of two cases

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