T-Cell Lymphomas | Primary Cutaneous T-Cell Lymphomas
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A major challenge in targeting T cell lymphomas is the absence of a unique marker to separate healthy from malignant T cells, avoiding live-threatening T cell depletion and CAR T cell fratricide. The peripheral T-cell lymphomas (T-NHLs) comprise a heterogeneous group of mature T- and NK-cell neoplasms. In the Netherlands, peripheral T-cell lymphoma not otherwise specified (PTCL NOS), angioimmunoblastic T-cell lymphoma (AITL), and anaplastic large cell lymphoma (ALCL) are the most common diagnoses, with an annual incidence of 1.14 per 100 The criteria for the diagnosis of B-cell prolymphocytic leukemia were also reviewed, and the group considered that the entity needs to be recognized only after rigorous exclusion of other lymphoid neoplasms, particularly transformation from CLL, mantle cell lymphoma (MCL), or splenic marginal zone lymphoma (SMZL).
Disease Overview: Cutaneous T-cell lymphomas are a heterogenous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fun- goides (MF) or Sézary Syndrome (SS). 3Division of Hematology/Oncology, Department of Internal Medicine, University of Michigan Rogel Cancer Center, Ann Arbor,
Primary Cutaneous T-Cell Lymphomas
Primary cutaneous lymphomas represent a group of extranodal non-Hodgkin lymphomas (T- or B-cell) primarily confined to the skin with no evidence of extracutaneous disease at the time of diagnosis (cf. secondary involvement of the skin). Mycosis fungoides is the most prevalent type of primary cutaneous lymphoma. Sézary syndrome, although rare, is a CAR-T (chimeric antigen receptor T-cell) therapy represents a revolutionary advance in treating hematologic cancers, offering promising outcomes for lymphoma patients, especially those with relapsed or refractory disease. Initially approved for diffuse large B-cell lymphoma (DLBCL), CAR-T therapy is expanding to address a broader range of lymphomas,
Molecular studies have revealed the genomic and transcriptomic landscapes of many T/NK-cell neoplasms, contributing to the organization and validation of the classification of T/NK-cell lymphomas and LPDs. Although the vast majority of CTCL subtypes are of the CD4+ T-helper cell differentiation phenotype, there is a spectrum of CD8+ variants that manifest wide-ranging clinical, histologic, and phenotypic features that inform the classification of the disease. CD8, like CD4, and cytotoxic molecules (including TIA and granzyme) are readily detectable via IHC staining
Peripheral T-cell lymphomas (PTCLs) arise from post-thymic lymphocytes and have heterogeneous clinicopathologic presentations. In the recent fifth edition (2022) of the WHO classification of hematolymphoid tumors (WHO-HAEM5), 34 mature T-cell and NK-cell neoplasms are grouped into 9 types, based on clinical and pathological features as well as latest Primary cutaneous T-cell lymphomas (CTCLs) encompass a clinically and biologically heterogeneous group of non-Hodgkin lymphomas (NHLs) defined by clonal proliferation of skin-homing malignant T lymphocytes and natural killer cells. They account for up to 75% to 80% of all cutaneous lymphomas. The current WHO-EORTC classification of
Peripheral T cell lymphomas (PTCLs) are a heterogeneous group of rare neoplasms. This Review outlines our current understanding of the genomic defects and host environment characteristics that
Over the past 30 years, the scientific community has made little progress in changing the natural history of peripheral T-cell lymphomas. Of the haematological malignancies, T-cell lymphomas have an extremely poor prognosis. One reason for this poor outcome has been that no treatment programme has ever been developed specifically for the broader category of the Currently, more than 20 different types of mature T-cell lymphomas (excluding primary cutaneous T-cell lymphomas) exist (Figure 1).
Peripheral T-cell and natural killer (NK)-cell lymphomas (PTCLs) represent a heterogeneous group of neoplasms derived from post-thymic T or NK cells, with diverse morphological patterns, phenotypes and clinical presentations. The International Consensus Classification and World Health Organization (WHO) classification of lymphoid and
Login to your ESMO Accountto sign up for ESMO newsletters and receive information about ESMO’s scientific and educational resources, events, member benefits. Moved PermanentlyThe document has moved here. The chapter discusses the cytological features and flow cytometry findings of. T-Non-Hodgkin lymphoma. In contrast to B-lymphomas, the T-lymphoma cases are challenging to diagnose by flow cytometry. The cytological features along with flow cytometry are needed to
Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). The diagnosis of MF or SS requires the integration of clinical and T-cell lymphoma is a fairly rare type of non-Hodgkin lymphoma. Learn more about the symptoms, causes, many types, and treatment and
T-Cell Lymphoma: Treatment Options Since there are so many different types of T-cell lymphoma, treatment varies widely. Standard lymphoma therapies may include chemotherapy, immunotherapy (like antibody drug conjugates) radiation, stem cell transplantation, and surgery. Letter to the Editor, Angioimmunoblastic T-cell lymphoma (AITL) originates from T follicular helper (TFH) cells and is widely considered to be an aggressive T-cell neoplasm. T-cell non-Hodgkin lymphomas (NHLs) are uncommon malignancies that represent approximately 12% of all lymphomas. 1 Various
Cutaneous T cell lymphoma is a group of lymphomas that initially manifest in the skin. This Primer summarizes the pathophysiology, epidemiology, diagnosis and management of these disorders.
In peripheral T-cell lymphoma–not otherwise specified and anaplastic lymphoma kinase–negative anaplastic large cell lymphoma (ALK – ALCL), patients with relapsed lymphoma demonstrated a superior median overall survival (OS) relative to refractory from the time of second-line treatment.
Mature T- and natural killer (NK)–cell neoplasms comprise a group of morphologically, immunophenotypically, molecularly, and clinically heterogeneous disorders with generally unfavorable outcome. Results of first-line chemotherapy are unsatisfactory for the most common T-cell lymphomas (peripheral T-cell lymphoma, not otherwise specified; Two decades ago, 2 groups independently described a distinct subtype of CD3 + CD56 + lymphoma 4, 5 characterized by extranodal distribution and an aggressive course. Originally classified as T-cell lymphomas, the development of monoclonal anti-CD3 antibodies showed that these lymphomas were in fact surface CD3 − and cytoplasmic CD3ε +. 6 Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma (PTCL), first described in 1981 and included as provisional entity γδ hepatosplenic T-cell lymphoma in the revised European American Lymphoma Classification in 1994. 1 After the identification of rare cases with an αβ phenotype, the term “hepatosplenic T-cell lymphoma,”
Disease Overview Cutaneous T-cell lymphomas are a heterogenous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS) Suchin KR et al. (2002) Treatment of cutaneous T-cell lymphoma with combined immunomodulatory therapy: a 14-year experience at a single institution. Arch Dermatol 138: 1054-1060 Peripheral T-cell lymphomas are a group of rare cancers of T cells or natural killer cells, most often with a poor prognosis. In recent years, significant progress has been made through the development of more specific therapies. This review aims to
Treating T-Cell Non-Hodgkin Lymphoma Non-Hodgkin lymphoma (NHL) is generally divided into main 2 types, based on whether it starts in B lymphocytes (B cells) or T lymphocytes (T cells). There are many different types of T-cell lymphomas, * Angioimmunoblastic T-cell lymphoma and follicular T-cell lymphoma have a follicular T-cell phenotype, but EBV + B-cell blasts may be present.
T-cell engaging–therapies have transformed the treatment landscape of relapsed and refractory B-cell non-Hodgkin lymphomas by offering highly effective treatments for patients with historically limited therapeutic options. This review focuses on the advances in chimeric antigen receptor–modified T cells and bispecific antibodies, first providing an overview of each T cell lymphoma, a type of non-Hodgkin lymphomas is a rare form of malignancy with poor outcomes.TCLS are a heterogeneous group of lymphoid malignanci
Purpose of Review Mature T/NK-cell neoplasms comprise a heterogeneous group of diseases with diverse clinical, histopathologic, immunophenotypic, and molecular features. A clinically relevant, comprehensive, and reproducible classification system for T/NK-cell neoplasms is essential for optimal management, risk stratification, and advancing understanding of these Lymphoma cells are transformed from NK- or T-cells, sometimes both. EBV-infection and subsequent genetic alterations in infected cells are central to NKTL development.
Peripheral T-cell lymphomas are rare and have poorer prognoses than more common B-cell lymphomas. It is therefore appropriate to highlight the current stat
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