Gamma-Delta T-cell Lymphoma: An Overview

Abstract

Gamma-delta T-cell lymphomas are very rare and aggressive T-cell neoplasms with complex heterogenicity and diagnostic complexity. Gamma-delta T lymphocytes originate from CD4− CD8− (double negative) thymocytes in the bone marrow and are distinct from alpha beta subtype. Four entities of gamma-delta lymphomas recognized by 2016 WHO classification of lymphoid neoplasms include: hepatosplenic Tγδ lymphoma (HSγδTL), primary cutaneous gamma-delta TCL (PCTCL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) and large granular lymphocytic leukemias (T-LGL). Extensive literature search based on small case series and case reports identifies few more subtypes of gamma-delta T-lymphomas which were not previously classified by World Health Organization. There remains a critical gap in our understanding of the subtypes of gamma-delta T-cell lymphomas and a lack of updated summarization. In this review, we summarize in detail on the classification, biology, heterogenicity, diagnosis, clinical behavior and treatment options of these rare but clinically important entities.

De Novo CD5+ Primary Gastrointestinal Diffuse Large B-Cell Lymphoma: Challenges With Treatment and Clinical Course

“…11,12 Clinically, this molecular subtype of DLBCL follows an aggressive clinical course similar to T-cell lymphomas. 13…”

“…11,12 Clinically, this molecular subtype of DLBCL follows an aggressive clinical course similar to T-cell lymphomas. 13 The role of surgical intervention in primary colon lymphoma was reviewed by Cai et al 14 have been studied previously involving surgery plus chemotherapy and chemotherapy alone for localized GI lymphomas. Early tumor stage, right-sided lesion, and DLBCL histological pattern seem to be the clinical characteristics of optimal surgical candidates.…”

Secondary Gamma-Delta T-Cell Lymphoma Not Otherwise Specified (NOS) From Chronic Immunosuppression

“…Gamma-delta T-cells make up less than 5% of peripheral T-cells and show tropism for various tissues, including the red pulp of the spleen, lymph nodes, and gastrointestinal and skin epithelia. GDTCLs are a rare subset of PTCLs that are clinically aggressive with poor response to treatment and poor long-term survival [1][2][3].…”

“…Gamma-delta T-cell lymphomas (GDTCL) are a rare subset of peripheral T-cell lymphomas that make up 1%-5% of T-cell lymphomas in the general population [1][2][3]. Most GDTCL fit into two subtypes described by the 2017 World Health Organization (WHO) classification of lymphoid neoplasms: hepatosplenic GDTCL, with associated hepatosplenomegaly and systemic symptoms, and primary cutaneous GDTCL with characteristic panniculitis and dermatological manifestations.…”

Gamma Delta T-Cell Receptor Lymphoma Causing Bilateral Pulmonary Embolism

“…It is an aggressive subset of natural killer T cell (TN/K) or natural killer cell lymphomas. TN/K lymphomas make up approximately 6% of all non-Hodgkin's lymphomas and PCGDTL comprises less than 1% of this group [1,2]. With only a handful of documented cases in the literature, and no documented cases complicated by pulmonary embolism, the clinical manifestations require further characterization.…”