Hepatosplenic γδ T-cell lymphoma: An overview

Koppany Visnyei; Michael L. Grossbard; Ilan Shapira

doi:10.1016/j.clml.2013.03.011

Hepatosplenic γδ T-cell lymphoma: An overview

Koppany Visnyei, Michael L. Grossbard, Ilan Shapira

Research output: Contribution to journal › Review article › peer-review

30 Scopus citations

Abstract

Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies. Among these, hepatosplenic γδ T-cell lymphoma (HTCL) represents an aggressive and treatment-resistant subgroup for which new avenues of treatment are critically needed. HTCL is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in hepatosplenomegaly and peripheral blood cytopenias. Another characteristic feature is the expression of γδ T-cell receptors. HTCL exhibits a rapid progressive course and an extremely poor response to currently known therapeutic strategies, with a 5-year overall survival rate of only 7%. In this review, we discuss the clinical, pathologic, and molecular characteristics of this disease, along with the challenges that are associated with its diagnosis and treatment.

Original language	English
Pages (from-to)	360-369
Number of pages	10
Journal	Clinical Lymphoma, Myeloma and Leukemia
Volume	13
Issue number	4
DOIs	https://doi.org/10.1016/j.clml.2013.03.011
State	Published - Aug 2013
Externally published	Yes

Keywords

Allogeneic transplantation
Autologous transplantation
CHOP
Hyper-CVAD
T-cell receptor
TCR

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title = "Hepatosplenic γδ T-cell lymphoma: An overview",

abstract = "Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies. Among these, hepatosplenic γδ T-cell lymphoma (HTCL) represents an aggressive and treatment-resistant subgroup for which new avenues of treatment are critically needed. HTCL is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in hepatosplenomegaly and peripheral blood cytopenias. Another characteristic feature is the expression of γδ T-cell receptors. HTCL exhibits a rapid progressive course and an extremely poor response to currently known therapeutic strategies, with a 5-year overall survival rate of only 7%. In this review, we discuss the clinical, pathologic, and molecular characteristics of this disease, along with the challenges that are associated with its diagnosis and treatment.",

keywords = "Allogeneic transplantation, Autologous transplantation, CHOP, Hyper-CVAD, T-cell receptor, TCR",

author = "Koppany Visnyei and Grossbard, {Michael L.} and Ilan Shapira",

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doi = "10.1016/j.clml.2013.03.011",

language = "English",

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journal = "Clinical Lymphoma, Myeloma and Leukemia",

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}

TY - JOUR

T1 - Hepatosplenic γδ T-cell lymphoma

T2 - An overview

AU - Visnyei, Koppany

AU - Grossbard, Michael L.

AU - Shapira, Ilan

PY - 2013/8

Y1 - 2013/8

N2 - Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies. Among these, hepatosplenic γδ T-cell lymphoma (HTCL) represents an aggressive and treatment-resistant subgroup for which new avenues of treatment are critically needed. HTCL is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in hepatosplenomegaly and peripheral blood cytopenias. Another characteristic feature is the expression of γδ T-cell receptors. HTCL exhibits a rapid progressive course and an extremely poor response to currently known therapeutic strategies, with a 5-year overall survival rate of only 7%. In this review, we discuss the clinical, pathologic, and molecular characteristics of this disease, along with the challenges that are associated with its diagnosis and treatment.

AB - Peripheral T-cell lymphomas are a heterogeneous group of lymphoid malignancies. Among these, hepatosplenic γδ T-cell lymphoma (HTCL) represents an aggressive and treatment-resistant subgroup for which new avenues of treatment are critically needed. HTCL is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in hepatosplenomegaly and peripheral blood cytopenias. Another characteristic feature is the expression of γδ T-cell receptors. HTCL exhibits a rapid progressive course and an extremely poor response to currently known therapeutic strategies, with a 5-year overall survival rate of only 7%. In this review, we discuss the clinical, pathologic, and molecular characteristics of this disease, along with the challenges that are associated with its diagnosis and treatment.

KW - Allogeneic transplantation

KW - Autologous transplantation

KW - CHOP

KW - Hyper-CVAD

KW - T-cell receptor

KW - TCR

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JO - Clinical Lymphoma, Myeloma and Leukemia

JF - Clinical Lymphoma, Myeloma and Leukemia

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ER -

Hepatosplenic γδ T-cell lymphoma: An overview

Abstract

Keywords

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